Some families spend their summer vacations at houses by the shore, or traveling to exotic places, and due to our addiction to social media we can all drool over our monitors wishing our friends could fit us in their suitcases. Last summer we started our own family summer vacation tradition, onwards to Philadelphia. Why Philly you might ask? Great history, amazing sights, fabulous museums, fun parks. But Chicago has all that. How about terrific cousins and my oldest friend in the entire world? That sounds cool, right? Alas, those are all the reasons we are lucky that we will be going to Philly every summer for a sweet vacation because Philadelphia is the home to one of the best Shriner’s Hospitals in the country, and more importantly the one that specializes in Aleck’s condition, Arthrogryposis Multiplex Congenita (gesundheit!). Hey, it could be located in Delaware (hi, I’m in Delaware), but we have to go there too. I’ll explain later.
Last year when we went to Shriner’s in Philadelphia we met with the Chief of Staff, an upper body pediatric orthopedic surgeon named Dr. Kozin. Between his extensive knowledge and experience working with kids with Arthrogyposis, and his bedside manner, we were hooked for life. This time we were able to coordinate the visit so we could meet the entire Arthrogryposis Team, the dynamic duo of Dr. Z for the uppers and Dr. VB for the lowers, in addition to meeting with Dr. Kozin again. Timing here was everything since Aleck’s lower body surgeon in Chicago has been preparing us for multiple surgeries to correct the turnout of his right leg and facilitate the growth of his pelvic bone which hasn’t been developing properly since his hip surgery 4 years ago. Her goal is to schedule surgery for this spring and with the waiting list to see Dr. VB at approximately 18 months we timed it perfectly. I don’t know if we’ll ever put Aleck under the knife again without at least a second opinion outside our current surgeon. There is truly just too much at stake moving forward and the solutions, as we’ve learned, are never cut and dry.
First we met with Dr. Z and his entourage. If there’s one thing that I find truly ineffective it’s cramming 9 adults into a room with one 4 year old and not one of those adults is paying the 4 year old any attention. Often there will be an occupational therapist (OT), to play and engage the child so that the doctor gets a clear view of their capabilities and so that the child is occupied while the parents listen, take notes, and answer questions. So of course Aleck started acting up during our conversation and by the time it was over we all felt frustrated. I had to ask Dr. Z often to put his medical terms into layman terms so I could even understand his recommendations and he seemed to find the way I framed treatments amusing which only added to my good humor. But enough about his lack of bedside manner, it’s already well published on all the AMC Facebook groups, time to focus on his recommendation. He would like to do a rotational osteotomy on both of his arms, cutting a small triangular wedge from his humerus to allow his arms to rotate up and out so that the palms of his hands are facing each other instead of always turned down. If you’ve ever seen Aleck “clap” you’ll understand, he “claps” by knocking his hands into each other but the palms don’t touch because of his inward rotation, this could correct that. There is no timeline on this surgery, we could elect to do it any time and at any age. On a scale of 1 to 10 for importance to an independent life, he gave it an 8 and clearly thought this rating system was quite amusing. (Insert Deep Breaths Here).
Next, we waited for over two hours to see Dr. VB. Even after they put us in a room it was almost another hour before the doctor came in. Thankfully they loaded us up with some bags of toys, a cool Star Wars backpack which is exactly Aleck’s size, and some other activities. Unfortunately we were still pretty frustrated from waiting so long and from our experience with the other doctor and his entourage. It’s not like we had much planned, we figured with a 1pm and a 1:30pm appointment we’d be able to see our friends for dinner and company, but as time ticked on we weren’t so sure. Dr. VB is the most sought after Arthrogryposis specialist in the country, and probably in the world, hence the 18 month waiting list. In fact, right now the waiting list for him to perform surgery is two years. Of course they move patients up if the surgery is time sensitive, but if it’s not then you need to really be able to plan in advance. His vast knowledge and experience isn’t the only thing that has them lining up in the waiting room, it’s also the way he attends to his families, his bedside manner. He speaks in clear English, he takes his time, he sees you himself, not with a lot of people around. He even draws diagrams and encouraged me to take a picture of it so I could really see for myself what he recommends for Aleck. In short, he wants to do the same thing to Aleck’s femurs as Dr. Z wants to do to his humerus. He wants to cut a small wedge out of each femur to rotate both legs and both hips better into their sockets to allow the balls of his hips to put pressure on the pelvic bone. That pressure will then stimulate the growth and development of the pelvis. Importantly he wants to have this done to Aleck in the next two years. And on a scale of 1 to 10, it’s a 10.
We made it to dinner, didn’t arrive until almost 7pm and they have two boys, one year younger and one year older than Aleck. I plowed through so much wine I was a bit embarrassed but it felt great. And then we went and cancelled our appointment with the upper body surgeon in Delaware. Neither Craig nor I could face another very long day of doctors and we wanted to do something fun. Plus, a great deal of the exhaustion and frustration of seeing these surgeons is that each one will measure Aleck’s range of motion. Often that means holding him down while he screams and someone bends his limbs while taking notes. Often that means I’m the one holding him down. Every single time it tears away at my insides leaving me feeling so sad and so spent, feeling almost mean putting him in these positions where the people that will help him the most are also the people that will hurt him the most. Plus we were already schedule to see Dr. Kozin two days later, so we decided that two upper body orthopedic surgeons in three days was probably enough.
The following day we enjoyed our morning at Reading Terminal, eating our way through every stall, and then made the trip out to Neumors DuPont in Delaware (hi, I’m in Delaware) to see Tariq, the creator of Aleck’s upper body exoskeleton, the WREX. It’s a stunning hospital only 45 minutes away from Philly, a terrific facility, and Tariq greats us like old friends, even entertaining Aleck by giving him a ride in one of their red wagons. Aleck tried on the WREX, they made some adjustments and then figured out what needed to be altered on the body of the WREX so that it will fit him more comfortably. The OT, Charlie, was a delight, and gave us a tour of their pediatric outpatient unit which makes the RIC look more like a 3rd world country (new one coming this spring!).
Now we only had one more doctor’s appointment to go, back to Shriner’s the following morning to see Dr. Kozin. We barely waited, he had no entourage, and only spent a few minutes and a few short tears getting Aleck’s measurements. Mostly we all sat back and just let Aleck do his thing, try to open doors, push buttons, and even coming to rest on the stool, his elbow bent by the pressure and almost to his mouth. Dr. Kozin’s recommendation for Aleck right now…don’t do anything and come back next year. He’s made huge progress since he saw him last year and he’s clearly using life as his own form of therapy, constantly trying to reach and do more and more everyday. Whew…yeah!
But now come’s the hard part. How to have this next conversation is a tricky situation. It’s time to talk to our lower body pediatric orthopedic surgeon here at home who we adore, both as a doctor and as a person, about the next steps. Our plan right now is to openly discuss Dr. VB’s recommendation for surgery and get her thoughts. Craig and I are fairly confident we want to go with his recommended surgery, but would she want to do it? She knows Aleck best, does she think it’s the right decision. To us it sounds like the most logical surgery, a rotation that would help correct a bit of the turnout but more importantly stimulate the growth of the pelvis in a more natural manner. It may not work and he may still have to have pelvic surgery later on, but we’d like to give it a shot without alienating her or severing our relationship. Having the surgery done in Chicago would be much easier on everyone since it’s 3 weeks in a cast, then back for X-rays, then another 3 weeks in the cast followed by therapy to get him back on his feet. But if she’s opposed, hey, thank goodness we love going to Philly.
In addition to eating and running like crazy at the Reading Terminal we hit some other fabulous sites. Did the tour of the U.S. Mint, which Aleck loved, had dinner on South Street and ate the biggest piece of pizza I’ve ever seen, which means a lot since I’m originally from the East Coast. Played games in Harbor Park, saw the Liberty Bell, visited Ben Franklin’s grave, ran the Rocky Stairs at the Philadelphia Art Museum (video coming soon), and spent a few hours in the Franklin Institute watching blood cells work their way through the human body. Since we drove from Chicago to Philly, we stopped in Pittsburgh on the way there and on the way back; discovered different neighborhoods, learned all about the “Pittsburgh Sandwich” at Primanti Brothers, and checked out the Strip District which we loved. Of course the icing on top was that we celebrated Craig’s birthday in Philly, found an awesome place that put a candle in his milkshake and even sang to him. Then we made one more stop on the way home in Oberlin, Ohio. Aleck insisted we visit their art museum, which was actually quite impressive. We walked around the town and enjoyed a lunch far superior to the usual rest stops. All in all we made the trip into a vacation, not just a series of doctor’s visits. We chose not to stay at the Ronald McDonald House, surrounded by families dealing with sickness and surgeries, and opted for our favorite low budget hotels instead, adding to that feeling that this was all part of our summer vacation. On social media it’ll look just like a typical family road trip and when Aleck talks about his summer vacation he barely mentions Shriner’s at all. Mission accomplished.