Hopkins Recap – Fall 2019

Happy Fall, Y’all!

Last month we made our biannual trip to the Johns Hopkins Greenburg Center for Skeletal Dysplasia to see Sam’s specialists. The 7-hour drive was a lot easier this time because we didn’t have to stop quite as often to feed Sam, and he was able to keep busy in the car for longer stretches with his toys and books. We even made a couple of stops at playgrounds so that Sam could burn off a little energy before hitting the road again.

This trip we were fortunate enough to be able to stay at the Ronald McDonald House in Baltimore, which was such a blessing. Not only was the facility beautiful, but it was only a few minutes from the hospital. Each morning breakfast was provided and each night when we returned from a long day of appointments, volunteers were serving dinner in the dining area. We’re so grateful to have had some of the financial and logistical burden lifted during our stay with RMH. They provide such a haven for families, especially those with children enduring long-term hospital stays. So the next time you’re at a McDonalds, consider donating your change to Ronald McDonald House Charities on Sam’s behalf!

Now on to the appointment details… Here’s a rundown of what his doctors had to say:

Monday: Orthopedics & Genetics
Our first appointment was with Sam’s orthopedic surgeon who took new x-rays of his spine and did a physical exam. Unfortunately Sam’s kyphosis has worsened since our last visit – something we had anticipated since he spends more time sitting and upright than he did six months ago. Because of this, the doctor prescribed a back brace to slow down the progression: the goal being to delay surgery for as long as possible (years, hopefully). You can see in his x-ray below just how curved his spine actually is.

Even though a brace will mean new challenges and adjustments for Sam in terms of mobility, we have felt for a long time that he would benefit from having one.

After we finished at orthopedics we headed over to see Dr. Hoover-Fong, geneticist and director of the skeletal dysplasia clinic. Her general assessment was that Sam is doing very well! During the appointment he was standing with our assistance and cruising along our chairs, and she was so happy to see how far he had come in his physical and cognitive development. She said she agreed that a back brace was the right call in order to prevent compression on Sam’s lungs, and that we’d wait to hear what the neurosurgeon had to say about the spinal stenosis in his neck and possible spinal decompressions surgery. She encouraged us to keep working with his therapists to further his development and continue to feed him a balanced diet to keep him from gaining too much weight on his smaller frame.

Tuesday: MRI & Neurosurgery
Tuesday was a very. long. day. We knew that Sam was scheduled to have an MRI under anesthesia that day, and as a result we had to stop feeding him milk and solid foods by 6am. So we all woke up 5am to have breakfast and then wait until it was time to head to the hospital for Sam’s 10:30am scheduled arrival time for the MRI. At about 10am the hospital called to inform us that radiology was running behind and that our new arrival time was not until 12:30 but that Sam still couldn’t have anything except a little water. Sam was already signing “eat” and “more” to tell us he was hungry, so we improvised with a distraction – a quick visit to the National Aquarium. Our tactic was only partially successful, as Sam kept signing “eat, more” in between glimpses of fish, sharks and eels. Meanwhile, because his MRI was being pushed back so far, we were going to miss our appointment with the neurosurgeon scheduled for that afternoon. So instead, the doctor agreed to come to the PACU after Sam’s MRI was complete.

To cut this story short, it was a LONG afternoon of waiting with a ‘hangry’ toddler – Sam’s MRI didn’t end up being until 3pm. We were able to be with him as they administered the anesthesia and stood on either side of him. It was incredibly difficult to see how scared he was and how much he fought it at first, but he was asleep quickly and then we were taken to the parents’ waiting area. We hadn’t eaten since 5am either, so we went to the cafe and had a sandwich and passed some time. By 4:30 the nurse came to get us to tell us he was awake and everything had gone well. We had been so worried about him and wondering if he’d be scared when he woke up, but when we stepped into his PACU room, he was cheerfully sitting on a nurse’s lap eating Goldfish and watching Sesame Street, still feeling the effects of the anesthetic I’m sure!

We waited a little while longer, fed him some more snacks and milk, and then were cleared to leave as soon as we spoke with the neurosurgeon. When she arrived, she reviewed his MRI with us and determined that no surgery would be needed at this time. Sam isn’t exhibiting any major symptoms related to the narrowing of his spinal cord (stenosis), and there is only a slight chance that his sleep apnea would be improved with surgery. Spinal decompression surgery carries a significant risk of increased neck instability, so she said it would be best only to operate if we notice any regression in his development.

We felt so relieved that Sam would not be needing any major surgery in the next few months, and we were very happy with how thorough the neurosurgeon was with us. So after all that, we packed up our loopy boy and headed ‘home.’ It was almost 7pm by the time we got back to the RMH, and we were thrilled to see that volunteers had brought in Chic-Fil-A for dinner!

Wednesday: Pulmonology
On Wednesday we checked out of the Ronald McDonald House and headed just outside of Baltimore to one of Johns Hopkins’ pediatric pulmonary clinics. The pulmonologist discussed Sam’s sleep apnea, CPAP use, and pending adenoidectomy with us. Since we found out the day before that spinal decompression surgery was not currently being considered, her recommendation was that we move forward with the adenoidectomy with Sam’s ENT here in Dayton and do another sleep study a few months later to see whether there was any improvement.

Then we finally started the trip home on Wednesday afternoon. We spent two nights in Pennsylvania just to relax and unwind as a family. While we were there we spent a day at the zoo and Sam really loved feeding the animals. We laughed about how even though he saw a lion, tiger, and bear, his favorite animals were the ducks that followed us around! During this two days, Sam also started saying “Mama” much to our surprise and delight! Our final stop of the trip was in Columbus to see Sam’s friend Clara who is the same age and has the same genetic condition. We have really connected with Clara and her parents, not just because our families share similar journeys, but on a personal level as well and we’re so grateful for their friendship.

The last time we were in Baltimore, we were grappling with the new reality of Sam’s diagnosis. It was so fresh and raw, that I don’t think we were able to see past those difficult moments. But this time around, I felt a glimmer of hope and empowerment that we’re doing everything we can for him – he has an incredible care team behind him and a whole lot of people praying for him and for us. It’s a relief that we don’t have to worry about major surgery at least for several months, so for now we just get to enjoy watching Sammy be a toddler.