Congratulations! Welcome to the OI family - you’re in good company here. I field a lot of messages on my Instagram from new and expecting moms with babies that have a more severe form of OI like Jackson does, and it is often the same conversation over and over. I recognize that not everyone feels comfortable reaching out, so I’ve put together a guide that would’ve been immensely helpful to me in the early days. I don’t consider my account to be an awareness account, but I have walked this road, and I know how important it is to share what we know with each other.
Resources
Share with your NICU team: OI Foundation Town Hall - Dr. Bober (Director of Skeletal Dysplasia at Nemours Alfred I. duPont Hospital for Children) Presentation on Care for the Child with Moderate to Severe OI in First Year of Life - Youtube Video Here and this study on OI in the neonatal period - Comprehensive pain management strategy for infants with moderate to severe osteogenesis imperfecta in the perinatal period
Join the FB Group: Expecting OI Parents - New members are screened carefully as medical information is shared and discussed within the private group. You’ll talk with experienced OI parents, gain advice from their lived experiences, and get support you need.
OIF Handling Guide: The OIF has many resources including this handling guide to learn from.
OIF New Diagnosis Toolkit: Another great resource for new parents navigating an OI diagnosis with their child.
Pregnancy + Diagnosis in Utero
We got the diagnosis for Jackson around 28 weeks. We were told he may live or he may not live, and that was a really difficult thing to navigate. Every meeting, appointment, and phone call was laced with a lot of heartache and emotional weight. It made it difficult to prepare for a future that included a living baby, and I had to learn a lot really quickly once J was born.
We saw a lot of the type 2 qualities I googled online, minus a softer skull (which many type 3 severe infants have as well) but his position in the womb made it hard for us to tell how soft his skull was. When J was born, the back of his head was like a big soft spot. None of these things meant he wasn’t going to survive the perinatal period. We also frequently checked his TC/AC ratio (I requested it each time - I even had one traveling MFM tell me, with a stern and sad voice, that his chest was “small.” Of course it was small! HE was small. The issue was if it was proportional for a baby with skeletal dysplasia.) and it was between 0.72-0.78. According to Dr. Bober’s presentation, below 0.6 can be incompatible with life. Size of his chest aside, we didn’t know until he was born how hard his chest wall would be. That is nearly impossible to tell in utero, and every OI baby is so different in how they handle respiratory function. We just had to wait and see.
A few other things that we saw on ultrasounds: Bones that would literally change shape in a few weeks time (from fractures), rib fractures, bowed and shortened femurs, a head that changed shape (wormian bones - they’re wild in utero sometimes), and the cutest little profile of all time. None of this meant Jackson wasn’t going to live. It pointed to a more severe case of OI, and that was all we knew at the time.
The NICU Stay
Every baby with OI is so different. We have been really lucky to meet so many other families with babies and kids similar to Jackson, but no two kids are exactly alike. One baby may have had issues with feeding but stable respiratory, and one may have been a strong eater but needed a lot more respiratory help early on (likely from pain after birth). Every single situation is unique, and knowledge is power. If you have a baby on the severe spectrum that needs more respiratory support, you’ll likely be talking about lungs more than bones. Please remember that I’m not a medical professional, and this is just advice based on what I’ve seen in our own child’s history. Let’s get into it.
I shared early in the post that Dr. Bober, a specialist top in his field at one of the top hospitals for rare skeletal dysplasia in North America, has a presentation on Youtube (given at an official OI Foundation Town Hall) regarding the NICU experience for babies with moderate to severe OI. You’ll want to start by watching his video here. Share with your team, take the role of educator, and be prepared to be as involved as possible. OI is a rare condition, and often the more severe cases require a multi-disciplinary approach to getting babies ready for home.
I like to (darkly) joke that the NICU stay with OI is a baptism by fire. Handling our babies is a learning curve - from diaper changes to appropriate PT intervention. Many hospitals don’t feel comfortable letting parents hold their babies early on (we did hold, but felt more comfortable doing so with a pillow). You have to summon a lot of inner strength to speak up, ask questions, and advocate for what your child needs. Make sure the NICU team posts bed signs that state baby’s fragile status for their own safety.
You’ll find early on that NICU’s are very focused on weight gain and feeding goals. Remind them that OI babies grow much slower. Jackson has always taken less food by tube or mouth, and he is a very slow grower. At one year-old, he’s still in 3-6 month clothing just barely. Our OI nutritionist wanted him gaining 5-10 grams a day, and recently it has been changed to 2-5 grams per day. Smaller tummies get full quickly, defective collagen can make the body digest slower/have more sensitivity, and if there are fractures healing (especially with pain meds on board) they will likely digest even slower.
Fractures are often lightly wrapped with soft cotton or ace wrap just to immobilize them. We were told babies heal relatively quickly at that age. The biggest issue is finding the balance between pain not affecting respiratory and pain medications not suppressing respiratory. We weaned from morphine by day 4 or 5 of birth, and continued with Tylenol PRN.
Finally, we made sure to get his first infusion before he left the hospital. We did Pamidronate and some do Zole, but regardless it’s shown to have great results in pediatric patients and is now a common practice to start right away in moderate to severe OI babies.
Clothing
We honestly didn’t use clothing until Jackson came home! We stuck with swaddle blankets only in the hospital, and I really liked the bamboo and stretchy brands the most. Jackson was a SWEATY newborn, and still is (thanks to the helmet). They have a harder time regulating temperature, and it’s just a thing. Lighter swaddles were a good fit because it kept him nice and contained if I was holding him (no loose limbs kept us from accidental handling fractures). When he came home, we started using clothing more frequently.
You’ll want smaller sizes only (Jackson wears 3-6 at 14 months old now) in newborn and 0-3. Light fabrics again keep them more comfortable, especially ones like bamboo that wick moisture. I avoided footies because of the pulse ox, but it is a lot easier to cuff the legs for shorter limbs too. Most importantly, you’ll want to have completely button down or kimono style early on. You may get brave and try some other styles - that’s great! We have slowly ventured to other styles after he’s grown and gotten stronger. Pants were not our friend until he was closer to 8 months old. I have found companies on Etsy (I’ll link the one below) that make extra stretchy shorts (great for older babies and toddlers rocking a more age-appropriate outfit) and pants with no elastic and lots of snaps. You can even make your own clothing with snaps or commission a seamstress friend to help. I made my own pants, and they were cute, but I defer to Etsy now for Jackson’s pants.
Kyte Baby - all bamboo: snap rompers, sleepsacks with a double zipper, swaddles
Kate Quinn - kimono onesies (new drops are categorized by fabric), panda pants (bamboo and size up for a stretchy OI friendly style), swaddles, union romper
Lou Lou & Co - stretchy light blankets in fun patterns
Paisley Prints Spokane - has a pattern for OI friendly snap pants (need to message for special order)
Doodle & Jack - shorties with maximum stretch and a well fitting length
Bathing
We were told at our first ever OI clinic that a fantastic way to get both PT and bath time done together was to lay some towels down in the tub and fill up a few inches of water. Jackson loves his tub time, and I think it relaxes his muscles and feels nice to have some weight relief on his body. Some OI parents use this tub religiously, and we have it also - set aside for travel. I recommend bringing home any chucks pads you can get from the hospital for sponge baths, as well.
Feeding
We came home with the Dr. Brown’s bottles and the ultra preemie flow nipples. When you’re in the NICU, often speech therapy will bring different nipples to try. This ended up being a good fit for us. We have OI baby friends who love the Como Tomo bottles as well.
The Peg Perego highchair is my favorite for feeding now that we’re working on purees. It reclines to 5 different positions, rolls easily from room to room, and even changes height. I pad everything Jackson sits in for support - you’ll notice in the picture below a few blanket rolls peeking out that support his neck and spine.
If you end up needing a feeding pump, we have found the Flying Squirrel to be an absolute LIFESAVER for moving room to room and for appointments.
Toys
When we buy toys for Jackson, I like lightweight ones! Early on, we hung them from a play gym with plastic links. You can hang almost anything from the links, and if you get a portable gym (like the one we have) it’s easy to move around with them. Now we have a bigger variety of toys including interactive ones. I’ve linked some of our favorites below!
Seating/Support
We are very cautious about how much support Jackson’s spine gets in everything from high chairs to seats to strollers. The best seating support has a variety of reclined angles to adjust with him as he grows. We did not push sitting him up early, and followed his lead as he tolerated. Often I pad his seats with a gel pad (from the NICU) and folded swaddle blankets to support him well. I recommend getting PT involved when you’re home to be a helpful resource - they were wonderful with tips for positioning Jackson safely and comfortably in different ways. Below are some favorites for seating/lounging at our house:
Nap Nanny: This is a favorite of many OI parents because of its supportive but comfy foam structure. No longer available due to recall - if you find one through a friend or through resale please use responsibly
Travel
The hospital sent us home with a car bed that Jackson has almost outgrown now, and we are in the process of getting him the next size up through insurance. We purchased a Nuna Pipa carseat that was highly recommended to us by other OI parents, but he tends to be more comfortable in long car rides (specifically for clinics in Omaha and Kansas City) laying flat. Protecting his neck and spine are a major concern. I would recommend waiting to get a carseat until your child is born to get a better idea of what they can tolerate.
Break Kit
Every OI parent has a different approach with their kit, and in the early days you may not need the full amount of supplies. Although, if you’re nesting and gathering, I recommend just preparing with everything on the list so it’s there for a rainy day. We used simple ace wrap in the beginning. You want enough supplies on hand at any time to immobilize fractures. We carry our break kit with us everywhere, and we also have a home supply stash organized and ready to go.
Overwhelmed? You’re Not Alone.
Honestly, I know this is a lot to read through. We were in the NICU for 8 weeks, and during that time I was able to slowly prepare for Jackson at home. Many of the things we needed were gathered slowly. I had to organize medical supplies from multiple DME’s (durable medical equipment) for feeding and oxygen, and the focus was there. Don’t feel obligated to buy everything at once. Take your time, learn everything you can about moderate to severe OI, reach out to others in the community, and prepare to educate your team on what is needed for the initial NICU stay. You are capable, you can do this, and your baby is going to be so loved.
