Omaha 2018

Did you catch that title? “Omaha 2018.” No subtitle. In contrast to our four trips to Omaha in 2017, my hope has been that this would be our one 2018 Omaha excursion – but I knew that would largely be determined by what we found out during this trip.

About a week and a half ago, we loaded up our minivan and packed up our family of six and drove out to Omaha, where we were overdue for FangFang’s annual OI clinic visit. World-class practitioners in multiple specialties related to osteogenesis imperfecta (OI) work together in Omaha to provide a clinic experience that, to my knowledge, is unmatched.

We arrived on Tuesday night, and FangFang and I headed to the hospital on Wednesday for the testing that would give us valuable information about her growth, her bones, and her body in general. This year was a light year in that all we needed to do was a dexa scan (which measures bone density) and a collection of x-rays (that check the status of the rods she currently has placed in both femurs, both tibias, and her left humerus; show us the status of her spine; and generally look at how her bones are growing and whether there have been significant effects from any recent fractures).

The highlight of the day for us was seeing FangFang’s former foster sister, Xiao, whom she knew in China before we ever met her. Our families have stayed in touch since the girls have come home, and we hope to continue to be able to schedule their clinic visits together and maintain this relationship for them. Our children from China have so little from their pasts – these connections that we can help them keep are so special. And in each other, they each have a friend who truly understands, who is living life as a Chinese adoptee with OI. Those connections will likely be invaluable to them as they grow and begin to negotiate the world with increasing independence.

We’d actually hoped the reunion would be even larger. There is a third sweetheart, Gabby, who lived at the same foster home as FangFang and Xiao in China, and she recently came home and was scheduled for her first clinic appointment at the same time as us – but unfortunately, her older sister (also from China, also with OI) broke her femur the week before clinic, and their family was unable to travel ☹ That was such a bummer – we’d been so looking forward to seeing all of them! There are a few other families with kiddos from China who have OI with whom we hope to continue to maintain connections, as well. We’re so thankful for these sweet moments between Xiao and FangFang – even at 3 and 4, they delighted in seeing another child like them, using a wheelchair, having scars from rodding surgeries, occasionally sporting a splint – we’re going to do all we can to continue to facilitate these connections for our kiddos!

While FangFang and I did that testing, Matt took our other kids to a park, and once we were done, they came back for us and we all spent the rest of the afternoon playing at the park.

In an attempt at frugality, I’d booked all six of us in a standard hotel room for our time in Omaha. While it was certainly frugal, it was also rather miserable. We were all on top of each other all the time, the kids had no room to run around or play, and it was just generally an unpleasant situation. We ended up spending most of the waking hours during which we were at the hotel letting the kids watch tv, because it was our best strategy to keep the peace. And now we know. We can certainly handle standard hotel rooms for a one night stop or something of that sort, but for any extended stay, it is unwise!

We debated how to handle Thursday morning clinic – whether all of us should go, so Matt and I could both be present for all of these doctor meetings or whether it would be better for him to take the other kids to do something more entertaining, and FangFang and I could focus, undistracted, on our conversations. It would have been great to have both of us there for all of our conversations, but ultimately, we realized that as the researcher and doctor-appointment-attender parent, I was probably going to be negotiating 95% of those interactions, anyway, while Matt parented our kids, and it would probably be easier for him to parent them somewhere other than a hospital room 😉

FangFang and I were at the hospital by 7:45, and we got to chat a bit more with Xiao and her family before clinic started.

After a nurse got FangFang’s height and weight, we were taken to a room that would be our base of operations for the rest of the morning while doctors and other providers rotated around to talk with us about their individual areas of expertise as they related to FangFang.

First up, we saw Dr. Esposito and Dr. Wallace, the orthopedic surgeons. I knew that their assessment of how her bones and the rods she’s had inserted into many of them (both femurs, both tibias, and her right humerus) would largely determine whether we needed to make a planned return trip to Omaha any time this year – and, thankfully, they don’t believe that will be necessary! Of course, we may end up back there anyway – a significant fracture requiring surgery would mean a drive to Omaha for Dr. Esposito and Dr. Wallace to operate – but we at least don’t need to plan anything now! Her left femur rod is the one they have the most concern about. It was the earliest placed, and the surgery was done in China, and it will likely be the first to require revision, but they said that as long as she isn’t experiencing pain or limping, we should leave it alone. We looked at her spine, and her scoliosis is not particularly severe, and the wedging we can see on x-rays has improved in the last year, largely due to the Pamidronate treatments she receives. Essentially, everything looks pretty good from an orthopedic perspective!

We also met with a researcher for a 5-year longitudinal study being done out of Omaha, in conjunction with other research sites, collecting data about individuals with OI to use in research studies, and we’ll have FangFang start participating next year. There is not a great deal of research available related to OI, and we want to do anything we can to be part of developing that, hoping for more and better treatments in the future.

The endocrinology team was very happy with the improvements in FangFang’s bone density shown by the Dexa scan. It’s actually a bit confusing, knowing what the level of improvement was – there is a discrepancy between what the 2017 report shows as her 2017 measurements and what the 2018 report shows as her 2017 measurements, and no one was quite sure why. But, regardless, her 2018 numbers show either a 15% or a 30-40% increase over her 2017 numbers, so we’ll continue with her same level of Pamidronate treatment.

We also saw a dentist and a dietician. The dentist continues to see no OI-related issues with FangFang’s teeth, which is great news. And the dietician talked with me about our diet and what FangFang eats and what her growth trajectory looks like, and she was happy with all that we’re doing, so no changes needed there.

The physical therapist was also very pleased with what FangFang is doing and what we’re working on with our local physical therapist, which was great news! The occupational therapist recommended an OT evaluation and maybe 4-6 sessions of OT at home to work on underlying core strength and skills – grip strength, endurance, screwing and unscrewing. I’m not thrilled to add likely another appointment to our weekly routines, but it’s definitely a good idea to address these things as early as possible, so we’ll see what we can do!

Overall I was very encouraged by the clinic visit, knowing that the Pamidronate treatments are having the desired effect, that her bone density is increasing, and that we likely don’t need to return to Omaha until next year for clinic. That’s pretty much the best report we could hope for!

After clinic, Matt took the younger 3 kids back out to play at a park while Miranda and I stayed back at the hotel room. She did some math and some art while I put in a couple hours of work and then napped. Another consequence of that whole six people in one hotel room arrangement was that no one was getting great sleep!

Friday was a really good day. We’d planned to meet Xiao’s family at the zoo, just to hang out and have fun, and that we did! Another family in town for OI clinic joined us, as well, which was great! They’d traveled all the way from the Bahamas for clinic, making our 5-hour drive look like nothing! The kids loved running around and the zoo, and we’d heard great things about the it, and it did not disappoint.

It was great to have this time to consult with these amazing OI-care experts and so good to connect with other families with kiddos who have OI. We were so thankful for our time in Omaha!

Surgery Update

I’d hoped to get a longer update posted last night, but it just didn’t happen – comforting FangFang when she was feeling yucky and then sleeping when she was comfortably sleeping took priority! I do so appreciate the prayers and support from all of you, though, so I want to let you know where we’re at today!

To back up a bit, we’d been planning for months to do bilateral tibia rodding surgery yesterday, but when FangFang broke her right humerus about 10 days ago, I’d sent a copy of the x-rays to her orthopedic surgeons in Omaha, just in case seeing those images changed their minds about surgery plans. Their assistant e-mailed me on Monday and said Dr. Esposito would talk with me on Tuesday about our options, but I didn’t think much of it. The fracture wasn’t incredibly severe, and operating on two limbs at a time is a fair amount already. However, when we saw him in pre-op yesterday, he suggested that we prioritize what was giving her the most trouble but try to rod all 3 bones if possible. He said he expected the humerus to continue to give her trouble (it had fractured previously in China and hadn’t healed to be as straight as would have been ideal, so we’d already been suspecting it could be problematic for her over time), and as long as it’s incapacitated anyway, we might as well fix it now and just have one surgery and one recovery period for all of it. These are the moments when I am most thankful we come to Omaha for care. Having an orthopedic surgeon who has worked with hundreds and hundreds of kids with osteogenesis imperfecta and can make these recommendations based on years of experience that give him more expertise than anyone else in his field is so helpful to me as a mom when I need to make the ultimate decision about what we should do. Though I was somewhat intimidated at the prospect of FangFang having 3 limbs fully incapacitated for a complete 4 week recovery period, I agreed that his proposal made the most sense, so that became the new plan for surgery, and they were, in fact, able to get to all 3 bones! They were even able to use the FD rods, which can expand with her bones as she grows, which is awesome!

It turns out that adding on the humerus does change the pain management aspect of post-op recovery pretty significantly, though. For rodding in the lower body, the preferred strategy for pain management is to place an epidural, but that epidural does nothing for pain in her arm. When she woke up, she was pretty upset. She got some heavy duty pain meds, all in quick succession, and some oxygen to help her after an intense crying spell, and that all helped.

She remained pretty out of it for most of the afternoon and evening, though. She felt nauseous and wanted to keep her bowl nearby…which was a bit awkward, since she cycled pretty rapidly through cycles of wakefulness and sleep, so she often fell asleep with her head in her bowl. We’d try to lean her back and remove the bowl, at which point she would awaken, outraged, and yell, “I need my bowl!” Whatever you say, my child…

As the afternoon wore on, she started to have some longer stretches of more peaceful bowl-less sleep, though.

And then she had more periods of wakefulness, during which she had very specific instructions for me about how I was to sit next to her on her bed with my arm underneath her head. That I can manage 🙂

We were able to FaceTime with Matt and the kids, too. She remained pretty out of it until later in the evening, though. She had a pretty awake, alert period from about 7:30 – 10:00 last night, in which she seemed increasingly herself, beginning to show off what she was watching on her ipad to her nurse and chatting a bit more.

We actually got a pretty good, long stretch of sleep last night – by hospital standards, at least 🙂 We all slept pretty well until around 4:00, when FangFang was asking if it was time to wake up and eat breakfast, but I assured her it was still night time, and we went back to sleep again. In my least favorite of standard-hospital-policy experiences, though, the lab person came at 5:30 a.m. and turned on the light and announced that we needed to get the nurse to come in un-tape the board under FangFang’s IV hand so she could do a blood draw. I’ve already asked what I need to do to request that the orders for tomorrow morning’s lab work be changed to be for once she wakes up instead of at 5:30. How institutions that are supposed to be committed to doing no harm came up with the brilliant idea of waking children who would greatly benefit from rest at 5:30 in the morning to stick them with a needle and draw blood is beyond me.

But now that we are up, FangFang is enjoying one of her favorite benefits of hospital time – virtually unlimited screen time 🙂

She is definitely more herself this morning. She still tells me every 5 minutes or so, “Mommy, I feel sick,” but she is more interested in engaging, more friendly, and chattier with people she deems friendly (yes to her nurse, no to the lab tech who drew her blood).

The pain management team, which I love here, has already come by this morning, and they just turned off FangFang’s epidural. Assuming she does well without it, they’ll pull it, which is the first step in moving us toward going home, a prospect to which we are all looking forward. FangFang has asked about 12 times in the 2 hours she has been awake when she can go home to her brother and sisters. I’m anxious to get home, too. Today is Atticus’s actual birthday, and I’m so sad to be missing it 🙁 Of course it phases him not at all – we celebrated his birthday last week, and when I told him last night that it was his last day of being 2 and then he’d be 3, he told me, “But I am 3 years old, Mom!” Okay, my boy 🙂

Essentially, before we can go, we need to get FangFang transitioned to all oral pain meds, get her next Pamidronate infusion (which can happen after the epidural is pulled), and get her orthopedic surgeons to sign off on discharge. I’m hoping and praying for tomorrow (Thursday) morning. If you’d pray with us to that end, too, I’d appreciate it!

Post-Surgery and Travel Update

Thank you so much to all of you who prayed us through our travels to Omaha for FangFang’s oral surgery and our return trip back to Missouri!

We left early Thursday morning and made it to the hospital just in time to meet the dentists who would be performing FangFang’s surgery the next day and do our pre-op consultation with them. After that we had the evening to ourselves, so we went and checked into our hotel…

…and then went out to dinner at Block 16, a hipster sandwich shop downtown, which all of us enjoyed! We tried to get FangFang a good last meal with all her teeth 🙂

She went to bed pretty well, and she actually did better than I thought she might with not being able to eat or drink after 8:00 AM. I woke her up around 7:45 to give her a clear liquid breakfast (jello and apple juice were her choices), and then I let her play with an iPad as a distraction while Catherine and I took turns getting some breakfast. We had a 10:00 AM check-in time at the hospital, so the morning was actually reasonably leisurely, and it wasn’t long before we were playing in the hospital playroom with brief breaks to consult with our nurse, a nurse practitioner, the dentists, and the anesthesiologist.

She was pretty happy right up until surgery. I actually declined Versed, and everyone seemed to think that was a good choice, because she seemed so comfortable and happy interacting with everyone, but as soon as she got about 10 feet down the hall from me, she started wailing, and they said I could come back with her. Her oral surgery was taking place in the procedure suite, which apparently has a lower standard of sterility than the OR, so I was allowed to walk into the room with her. I really wish all hospitals would do that for all procedures, whether they’re in the OR or not. FangFang is going to need a number of surgical interventions over her lifetime, and I’d prefer that, as much as possible, she see hospitals as places that help her, as opposed to the locations of traumatic experiences. Nurses seemed very concerned that it might be overwhelming to me to see her go under sedation in preparation for the procedure or have her throat suctioned afterwards, and they didn’t want me to feel uncomfortable. I assured them that I’d witnessed my husband experience cardiac arrest, so nothing they were going to do that day was going to make me uncomfortable, and if I needed to get out of the way, I’d do it. And most importantly, this is about FangFang, not me – if she’s more comfortable with me there, that trumps all else. They let me hold her and sing to her while she went to sleep, and I was so glad.

Catherine and I grabbed some lunch at the hospital cafeteria and then headed back up to our room to wait for FangFang. The dentists came and talked with us and said they’d pulled 5 teeth – the offending back molar that had the deep cavity giving her so much pain and her 4 front teeth, all of which had significant cavities. Because they’d pulled so many, they hadn’t needed to cap any teeth, but the crowding in her mouth will continue to make brushing and flossing a huge priority. They do not believe she has dentinogenesis imperfecta but that it’s more likely that we’re playing catch-up from her years in an orphanage, plus the crowding of her mouth, which is good news, because it means there’s some chance she won’t continue to have such serious dental issues.

It wasn’t long before I was allowed to go back to FangFang in recovery, and I walked in just as she was starting to open her eyes. She was in pain and angry. We got her Tylenol right away, and she wanted to leave that area, so we got to go back to our room right away, but she was still mad. We gave the Tylenol a bit of time to work, but it didn’t seem to be taking the edge off at all, so it wasn’t too long before we requested something stronger, and once she had a dose of Oxycodone, she started to calm. She cried for the mouth pain and cried in hunger and cried from her sore throat every time she had to swallow (she’d been intubated for the procedure). We started gradually introducing some clear liquids – apple juice and water and then jello, and she handled that well (no projectile vomiting!), and just before 4:00 they said we could go!

We weren’t sure how FangFang would do on the car ride home, and I was so thankful to have another adult with me who could help monitor her while we drove. She was pretty content watching Frozen and Daniel Tiger, though, and slept just a bit. She was even happy enough to try on goofy hats at a truck stop where we stopped to give her more pain meds and get gas!

We made it back home just before 11:00 last night, and she was very happy to be back, as was I!

Honestly, the trip itself went pretty well. That was really largely due to Catherine’s presence with us. I so enjoyed getting to chat with her on our drives – it was so much more fun than just driving by myself – and as a mom to four, it almost never happens that I get 10+ hours to hang out with a friend! And she was so helpful in assisting me with everything FangFang needed, getting juice or jello or washcloths to wipe up blood, and entertaining her while I talked with the doctors and dentists. I’m so, so thankful she came – such a blessing and encouragement.

And I’m so glad to be done with the procedure. FangFang was in a fair amount of pain yesterday but seems to be feeling a million times better today. She’s really been in pain for almost a month, and I’m so glad we were able to get this dental work done quickly and be done with it.

Re-entry is always rough, at least for me. I’m so excited to see everyone, but I’m also worn out. I really just want to have some quiet, alone time to read a book and relax. But there’s unpacking to do, and I’m behind on my work week since I was gone for 2 days, and kids need to be fed and cared for, and things at the house are just a little out of sorts any time I return from being gone. It always feels overwhelming to me, and I get snippy. There’s nothing that reveals your selfishness like parenting – and I think that’s doubly true when you add in any special needs. I do feel stretched, and I do feel tired, and I do feel overwhelmed at times, but that’s not a license to be unkind to anyone else, and I definitely fail at living that out.

I’ve tried to spend some time helping everyone settle back in. FangFang and I snuggled and read a book this morning, and a bit later Madeleine CaiQun and I got some one-on-one time reading on the couch together. Miranda and I had some chats, and Atticus came and snuggled with me for a while.

Matt has the kiddos out at a park right now, and I’m hoping to use this time well, doing some catch-up on all the tasks I need to tackle, but also to recharge and be prepared to love well when the rest of the family returns. I spent some time reading my Bible and praying and journaling, which has helped to settle my heart. I’m hoping that when everyone comes home, we can have an evening of enjoying being together, both in cleaning up the house some but also in just spending time together. These people have my heart, and I want to live that out, day by day, moment by moment.

Surgery Tomorrow Morning

Tomorrow morning we take an important step in this journey of living life with osteogenesis imperfecta (OI). FangFang is scheduled for bilateral femur rodding surgery. For those of you who would enjoy a detailed explanation, feel free to check out this link from the OI Foundation. The short version is that right now, her bone density is very low, and her right femur has significant bowing (curving). That means that if she were to try to pull to a stand (something she has been starting to attempt recently), chances are high that her femur would snap. Try to imagine for a moment the pain that would be involved in a significant break of this largest bone in your body, and you’ll understand why we’d like to avoid that scenario. Her left femur fractured about 10 months ago in China, and she had a rod placed in that leg at that time, but it will be replaced during this surgery, and her right femur will be rodded for the first time. These rods will act as internal splints, straightening the bones, giving them added strength and stability, and lessening the severity of any fractures that do occur in the future.

FangFang’s orthopedic surgeon recommended this course of action when we saw him in January, and it is the consensus of the other parents with whom we’ve spoken that it is absolutely the best choice for her. And so, instead of spring break on the beach or exploring a fun area nearby or just enjoying some quality family time at home, we have spring break: bilateral femur rodding edition.

My mom and FangFang and I made the 5 hour drive to Omaha this afternoon and got settled into our hotel room, where we hope to get some sleep before an early hospital check-in tomorrow morning.

Would you please pray for us this week as we tackle surgery and these first few days of recovery? In particular, these are some things for which we’d very much appreciate prayer –

  • that the surgery itself goes well. The surgeons performing her surgery are some of the very best surgeons in the world who specialize in caring for children with OI, and they have done this exact same operation innumerable times, and we have full confidence in them, but no surgery is ever routine when it’s for your child.
  • that we are able to manage her pain – both physical and potentially emotional – well over the next few days. Physical pain after this particular surgery is intense, and we, as well as the nurses involved in her care, will need to stay ahead of her pain with the best medications for her. Additionally, we’ve done all we can to explain what’s going to happen and read books and show her pictures, and I’m as confident as I can be for a 3-year-old who has been exposed to English for just over 3 months that she’s well-prepared, but it’s hard to know how much she understands. She’s going to wake up after surgery with an epidural and double leg splints. She’s such a happy kiddo, and I’m hoping she won’t be too distraught by her situation this week.

  • that my mom and I are able to comfort and entertain her well this week. She’s going to be in pain, and she’s going to have very limited mobility. We’re going to need to be creative and hands on in our parenting (and grandparenting) to care for her well, and while I’m hoping for some bits of down time, I don’t really know what to expect, and I know I need to be prepared for some long days and nights.
  • that Matt and our other 3 kids can have fun together during this week at home. Honestly, I think he has the harder parenting job this week, caring for 3 kids by himself 24/7 (except for brief breaks offered by a friend, for which I am SO thankful – having a couple hours to himself to run to the gym is going to make his job so much easier!).

  • that we can head home early. I’ve been told that if everything goes well, we can hope for discharge on Thursday or Friday, then they’d like us to spend another night in town, and we’d be able to head home the next day. I’d really like to be able to head home ASAP. I don’t enjoy being away from Matt and the rest of our kids, and I think FangFang will be much happier at home with her brother and sisters than stuck in a hospital room. Even tonight, as she was falling asleep in her hotel pack ‘n’ play, she repeated several times, “Night night, Atta.”
  • that we’re able, as a family, to care for FangFang well even as we return home. I really don’t know what these next few weeks will look like, and I want to be flexible with our daily routines and with school and with my expectations of what things will look like, and I hope we can all be selfless in our care for her during her recovery.
  • that the rods do their job well. FangFang very much wants to be able to stand, and we believe (and all the medical professionals with whom we interact believe) that having rods in her femurs will help her to do so safely, and we hope that is the case.

I’ll keep you informed as I’m able. Thanks so much for joining us in prayer as we take this step forward with our baby girl!

Heading to Omaha This Week!

Later this week we’re packing up and heading to Omaha for their OI clinic! Why, you might ask, would we do that?

When we were reviewing FangFang’s file, before committing to pursuing adopting her, we’d gotten in touch with our pediatrician, who reached out to one of the orthopedic surgeons in the area. He let us know what treatment would generally entail (surgeries and bisphosphonate infusions) and said that care could all be handled locally. And it likely could. So why travel? Why, in fact, change our insurance coverage to a different, likely more expensive plan, solely so that we could travel?

Osteogenesis imperfecta is an extremely rare condition. Approximately 25,000 – 50,000 people in the United States are estimated to be affected with OI – which means that in a country with a population of approximately 324,349,000, less than 0.02% of the population is affected. There is an OI Clinic right here at Mizzou, which, based on the most current data, sees…6 people per year. In contrast, in the same year, the clinic in Omaha saw 176 people. That number is still so low – but it’s almost 30 times higher than the number seen at Mizzou. The doctors involved with the OI Clinic at Omaha Children’s Hospital are recognized internationally as experts in caring for children with OI. Their research relates to OI, they speak at OI conferences, they consult with other doctors, and their expertise shines through when they interact with parents. As we began to research OI and speak with other parents of kids with OI, they almost unanimously recommended making the trip to Omaha and having these doctors involved in our daughter’s care.

Additionally, the clinic has a multidisciplinary approach. In just a couple days, we’ll be able to have FangFang do all the testing that is needed to give her doctors the information they need as they determine the best course for her treatment going forward, and see all of the doctors we need to see. We’ll do x-rays to get a good look at her bones and a DEXA scan to measure bone density. She’ll have an audio test (because our hearing is dependent upon the tiny bones in our ears, people with OI are more likely than others to have hearing issues), and we may do bloodwork and run some genetic tests. Then we will meet with a whole team of doctors and other medical professionals, including an orthopedic surgeon, an endocrinologist, an audiologist, a dentist, a physical therapist, an occupational therapist, a nutritionist, and a social worker, each of whom can speak to some facet of FangFang’s condition and give us insight into how best to care for her.

And this girl? In combination with these doctors? She’s going to kick some OI butt 🙂

ff-fierce

These are going to be some long days, but I am oh-so-thankful for the opportunity to go to this clinic and see these experts. We want to do everything we can to care for FangFang as well as possible, and that means getting her to the OI experts who can best advise us and help us to care for her. We’re excited to meet them, excited to hear their thoughts on a treatment plan for FangFang, and excited to get started doing whatever they recommend!