First Fracture? Yeah, Probably.

I was planning to write about something else today…but I think I should share about this instead. As a pre-adoptive mama to a precious little girl with osteogenesis imperfecta (OI), I did my homework. I talked to doctors, and I talked to other mamas. I prepared and traveled to China with a break box (a box of medicines and splinting supplies that would allow me to care for any fracture at any time). But I hoped I wouldn’t need it – not for a long time, definitely not while we were in China, and hopefully not until after our first trip to see the specialists at the OI clinic in Omaha. And I prepared myself mentally for the drama of it all. I’m not a stranger to medical trauma and the necessity of sometimes keeping a cool head, doing what needs to be done, and leaving the feelings for later. I knew it could still be tough, though – imagine a three-year-old fracturing their femur. Not all fractures are severe, but some are, and the probability of a severe fracture in a child with OI is significant.

We made it through China with no fractures, and I breathed a sigh of relief, and with our appointment in Omaha approaching, I was hopeful we’d make it until then without any breaks to any of FangFang’s bones. Alas, it was not to be. I’m pretty sure we’ve got our first fracture on our hands.

Remember that toy car FangFang so desperately wanted and was so thrilled to have?

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Well, she and it may have been involved in a collision with the other toy car last night. I knew that was a possibility when I ordered the car, and it did give me pause. However, what I’ve taken away from conversations with people with OI and mamas to kids with OI is that most of them would prefer to be allowed to play and have fun and risk the fractures, rather than being isolated and protected in a bubble.

After the crash, she cried and reached for me, and I held her and comforted her. We asked her if she had an owie, and she pointed at her lower leg, and once she calmed, Matt and I took a look at it. She let us look at and feel it, and she never winced as we palpated it. She sat on my lap and laughed at her siblings’ playing, and she saw her pajamas on the couch and asked to take off her clothes and put on the pajamas and didn’t have any strong reaction to pants going off or coming on. But she didn’t want me to put her down and did point at her leg a couple times. I checked in with another mama to a kiddo with OI, and her assessment from afar was that it was definitely not severely fractured but that it could be a micro-fracture or a slight crack, something that might not even show up on x-rays for a week or two until a callus has formed – or it could just be a normal bruise like any other child might get. If it were bad, we’d know, and we should probably try to make sure we didn’t let her do anything to further aggravate any injury there but otherwise just watch and see how she was doing. That’s what we did, and we had a normal bedtime routine, and she slept through the night just fine!

This morning she seemed a little off, though. She seemed a bit nervous to take off her pajamas, and instead of whipping her legs up in the air for her diaper change and then sliding them into her pants legs, she was picking up that leg with her hands. She’d say, “Mama,” and point to the same part of her leg that she’d been indicating last night. She was also asking for me to rub lotion on it. Poor girl, I think she may have thought that was the entirety of my repertoire for making things feel better! But she clearly felt like there was something going on that needed care. So…back to my OI mama friend I went, and her response this morning was that it really sounded like a minor fracture. That’s what I’d been thinking more and more, as well, and our leaning in that direction was strengthened when, as I was discussing the situation with my friend, FangFang began to attempt to splint the leg herself! Wow.

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I gave FangFang some Ibuprofen and talked through our options for our next steps with my friend and decided to take care of this one at home. We could have gone in for x-rays, either through the emergency room or calling the doctor who will probably be her local ortho, but there’s a good chance a small fracture wouldn’t have shown up on x-rays at this point anyway. Additionally, whether it did or not, it seemed clear FangFang’s leg needed a splint – for kids with OI, as my friend said, “If it quacks like a duck, then it is a duck, meaning if it seems like a fracture it’s probably a fracture,” and you need to treat it as such. I could do a splint quickly and easily at home, though. Beyond that, the biggest help doctors can offer is a prescription for stronger pain meds, but this one seems like it should be manageable with Tylenol and Ibuprofen. Plus she’s going to have a comprehensive set of x-rays next week in Omaha, and she’s going to be exposed to enough radiation in her life that I don’t feel the need to have a double exposure here in a situation where the x-ray would give us no necessary information.

We pulled out our break box and set to work on our first real splint.

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One blessing is that she’s already had her femur rodded in this leg, so we don’t have to do as significant of a splint. I believe the likely fracture is in her tibia, so the joints above and below need to be immobilized. For a non-rodded femur, we wouldn’t be able to stop the splint anywhere along her upper leg, though, or we’d be creating a site at high risk for a femur fracture, so we’d have to splint up past her waist. Thankfully, in this situation we can just splint through mid-thigh.

(For you OI mamas out there, her foot actually is at close to a 90 degree angle here; it just doesn't look like it because of the awkward angle of the photo!)
(For you OI mamas out there, her foot actually is at close to a 90 degree angle here; it just doesn’t look like it because of the angle of the photo!)

And here she is, all set and back to her usual happy self 🙂

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She seems slightly annoyed by the fact that the splint’s bulkiness and weight makes it a little harder for her to move around, but I’d rather face a bit of annoyance than a more significant tibia fracture! Overall she really seems completely fine, and I’m so happy about that! First likely fracture down with minimal trauma and with a cool bright blue splint of her choice! Now we’ll just hope we can make it to Omaha before we have any more significant trauma 🙂

Preparing to Parent a Child with OI: What Does that Look Like?

When I first heard of osteogenesis imperfecta (OI), I assumed that it just meant that the bones of the person affected by it would fracture more easily than those of other people, but as I shared here, there’s actually a bit more to it than that.

There’s also more to parenting a child with OI than I realized at first, and I’ve spent the last 9 months doing all that I can to learn about that. I began by talking to moms of kids with OI and adults with OI as we were reviewing our daughter’s file. Of course, I also talked with our pediatrician, who consulted with a local orthopedic surgeon, and I made contact with the orthopedic surgeon and endocrinologist who run the OI clinic in Omaha, offering some of the best care in the world for people with OI. All of that was incredibly helpful, and  I don’t want to discount the expertise of medical professionals, but OI is such a rare condition that very few doctors and nurses have experience with it, and I’ve so appreciated the opportunity to learn from the experiences of parents who are living right now the reality of life parenting children with OI.

One of the moms with whom I connected added me to a Facebook group of parents who had adopted children with OI so that I could ask questions there, and those mamas answered so many of my questions. And once we accepted Fang Fang’s referral and committed to moving forward with adopting her, I joined the Facebook group for all parents of children with OI, and that group is an awesome resource, as well. And these parents? They are amazing. Not only do so many of them take such excellent care of their own children, but they also have gone out of their way to pass on their wisdom to me (and to other expectant parents).

One thing I’ve learned from those parents is the importance of having a “break box” on hand at all times, whether we’re at home or out and about. Fractures can happen at any time, and we’ll want to be prepared. It’s true that we could go to the emergency room any time a fracture happens, but an ER visit is not always necessary, and even if it is required, we can do a lot to make our daughter more comfortable before transporting her. We should have medication available to address spasms and pain as a first line of defense. Then we should have splinting supplies, so that we’re able to immobilize limbs and splint any fracture at any time.

Another mama actually sent me a whole stash of supplies for the beginning of a break box, each labelled with instructions for use!

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You cannot believe how excited I was to get that box! I also started to add to it gradually as I learned more myself. There isn’t anything like a book you can read about how to parent a child with OI or even about OI itself, but I’ve gleaned so much wisdom from reading other parents’ posts in the Facebook group for parents of kids with OI, seeing what challenges other families face and what advice they receive.

This month another mom who sometimes travels through our area for work stopped by our house and gave me a whole morning of her time to talk about parenting her daughter who has OI and give me a hands-on splinting tutorial! What an amazing blessing! Fortunately, both Miranda and Madeleine CaiQun were willing to help us practice 🙂

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I’ve also learned about the need for a wheelchair – and specifically a wheelchair that is customized for her little body. Fang Fang is tiny, but she is 3, which means she’s at an age at which children are generally able to be mobile themselves. At home, she’ll be able to crawl or scoot, but that’s less socially acceptable at places like Target, and we’ll need to be able to facilitate her independent mobility. Additionally, we need to anticipate fractures. We don’t know when or how they’ll occur, but we do know they’ll occur, likely to major leg bones at some point, so we need to be prepared, and that means having a wheelchair available. We’ll also likely need a gait trainer and/or walker for her at some point, but we’ll wait for her physical therapist to weigh in on that.

I’ve been challenged to be an advocate for Fang Fang, even in medical contexts. I got a bit of a taste of that after Matt’s heart attack in February, but this will be more of a sustained need. Because OI is such a rare condition, I need to be an expert, and I need to be willing to discuss medical procedures and treatments with doctors. I need to ask that her blood pressure not be taken unless absolutely necessary, because the tightness of blood pressure cuffs can cause fractures. In case of fractures, I need to insist that I position her for x-rays, because well-meaning medical professionals who are not experienced in working with individuals affected by OI sometimes don’t know how to move limbs without causing further injury.

I’ve asked many questions of our pediatrician and of other OI moms, but I’ve also been seeking out other resources. I talked with the HR department at Mizzou to help me figure out which insurance plan would offer us the best financial coverage for all the needs we expect to have in the next year. There are two amazing mamas to kids with special needs who are part of our church, and both of them have been so gracious in answering my many questions. I’ve also talked with our local school system. And I’ve made contact with a local organization supporting individuals with disabilities. I have the information I need in order to get her set up with all the local support for which she’ll qualify as soon as she gets home.

By nature, I’m really a very dorky person, so I have actually enjoyed doing all this research and learning as much as possible about OI and how to care for Chen Fang once she’s home. Even if that weren’t the case, though, I believe I owe it to her to prepare as well as possible for her arrival, and I’m doing all I can to make that a reality!