We actually flew direct into Washington D.C. and then rented a car to drive to Baltimore. The flight went wonderful, Sam was comfortable but I could tell he was a little stiff once we exited the plane. We picked up our big rolling bag and headed out to the shuttle for the rental car. I noticed Sam was moving slower as he struggled to climb the steps on the shuttle bus. I began to pray that he would stay comfortable enough to make it on the drive to Baltimore. We arrived at the rental place and were told "We have unusually high rentals today and your SUV is not available." At this point Sam is beginning to show his discomfort and let the lady know that her answer was unacceptable. She could see our situation could possibly escalate quickly and she offered us a Jeep Liberty instead. I told her as long as the suitcase and wheelchair fit in the back and Sam can stretch out in the back seat we would be good to go. We were quickly on our way with Sam sitting sideways so he could stretch out his legs. Sam seemed to sense that a vacation was not in the picture and began to say continuously "Mommy, go home". Now take a moment and picture yourself driving in a new state, through two major cities, with a bucket load of worry and anxiety while your passenger is showing a mixture of pain, frustration and anxiety and is repeating the same phrase over and over. I quickly pulled off and reassured Sam that he was fine and I was right there with him. I gave him a snack and a promise of some soda (which in Sam's case is green tea) when we got to our destination. He settled in and continued to repeat his mantra...just at a lower volume and I took a few deep breaths and carried on.
We arrived at the Hackerman Patz House and Sam was thrilled when we got to our room and he could take off his shoes and lay on the bed and put himself in traction. The house is very nice and is set up like a home away from home. Amy greeted us and was extremely helpful throughout our stay. Since we were both tired we had an early dinner and called it a night. The next day we headed over to Sam's appointment. It was one of those times when you really don't want to go but you really want to get it over with.
If I'm perfectly honest I knew I wasn't going to like hearing what they had to say. It wasn't going to be something simple...and I was going to have to make some more really touch decisions on Sam's behalf. They ordered x-rays and Sam decided to show them he wasn't happy about it. After about 5 minutes of yelling and hitting the table I finally convinced Sam to climb on and we managed to get through them. As I waited for Dr. Standard to come in my mind felt like it was in auto-pilot, sort of a "been here, done this" kind of feeling. When you have gone through a major surgery with your child that didn't yield the hoped for results you become a skeptic. It is really hard to push all those doubts away and replace them with any semblance of hope. I want with every fiber of my being to see Sam walk with a reasonable gait and without pain. There was a time in Sam's life when I wanted him to be able to do everything any typical child can do, actually I wanted him to do them better than any typical child. I wanted him to dispel all the preconceived notions about Down syndrome and just be a superstar. As I looked at Sam in that waiting room, my heart over rode my mind and there sat "MY SUPERSTAR". Not the one I imagined but instead the one that has been formed as a result of the twists and turns on a journey that neither he or I could have ever imagined. Sam looked up and smiled and said "Love you Mommy, all my heart!!" I crossed the room, hugged him and said "I love you too Sam, with all my heart, forever and ever" and Sam said "Amen". I chuckled and reassured him that no matter what...we were in this together...and we would figure it out. I know that Sam is amazingly good at picking up on my feelings and disposition so I relaxed, opened my mind and heart and prepared it for whatever was to come.
Dr. Standard came in and immediately focused on Sam and then turned to me and shook my hand. Just that simple act of connecting with Sam first meant a great deal to me. He asked me about Sam's first surgery and when it was done. He asked me how Sam's current function is and I explained that Sam doesn't express pain like the rest of us he just adapts. He wakes up in the morning and says "Well, this is what it feels like to be Sam Mayer today" and he adapts, he puts himself into traction, he crawls instead of walks, he remains in bed instead of moving around. Dr. Standard then asked about Sam's pulmonary issues and wanted to know how he came through the last major surgery. I explained that he spent an evening in the ICU after surgery to be monitored and then came home with oxygen.
Our conversation then turned to how he assesses a new patient and if a hip or situation is in the salvage mode or rescue mode. The salvage mode is the last step and consists of procedures like a hip fusion or total hip replacement. In the treatment of Perthes the best outcome is based on 3 things. The first is containment, the second is range of motion and the third is pain relief. Sam's first surgery was successful in keeping the hip contained but he still struggles with range of motion and pain issues. At first glance, Dr. Standard was considering Sam's hip to be in the salvage mode but as he studied the x-rays and thought of other similar patients hip structures he felt there was a strong possibility of saving Sam's hip. Arthrodesis or hip fusion is often used with young patients to provide a pain-free stable hip which can be later changed to a total hip replacement. It is also used when a total hip replacement has failed. In my research hip fusion results in a pain-free fused hip in 70% of patients. Recovery for this surgery is about 12 weeks and male patients tend to have a better outcome than female patients. Dr. Standard did not feel Sam was a candidate for a total hip replacement because of his age but more so because of the presenting age of his skeletal structure. In Sam's case if he were to look at his x-ray without any other information he has the skeletal bone development of a nine year old instead of an almost 12 year old, so his potential for bone growth is still very good. With a rescue approach Dr. Standard would like to create a somewhat spherical head in a somewhat spherical cup with good movement that would potentially give Sam 30 good years of pain free movement. In Sam's case the external fixator may need to be enhanced due to his size and strength. I asked about the viability of his femur when his current hardware is removed and more holes are placed for the external fixator. That femur bone is significantly compromised and I worry about him breaking it during the healing or rehab process. Dr. Standard explained that when he assesses Sam's hip during the beginning of the surgery process if he felt there was osteoporosis or a risk of a weakened femur he would put in a prophylactic femoral rod that would provide stabilization of the femur and could be removed at a later time. The external fixator would actually help in the process of removing Sam's current hardware in order to keep the hip in a good position and good support for the healing of the hardware holes.
Soooo....if we look at the procedure to save Sam's hip this is what it will consist of. Prior to surgery we would have Sam's pulmonary doctor talk with an anesthesiologist that Dr. Standard would choose specifically for Sam's case. The surgery in Sam's case will probably last 4-5 hours due to the need to remove hardware, possibly insert a femoral rod, apply the fixator and do a scar revision. The surgery is performed under general anesthesia with an epidural catheter placed while Sam is asleep. The epidural is essential for post-operative pain management and allows for physical therapy to begin immediately after surgery. Sam would be moved to the ICU for a couple of days to monitor his respiratory functions.
The surgery consists of soft tissue releases (tendon releases) in the groin and inner thigh, an arthrogram of the hip joint, a small diameter core decompression and bone stem cell graft injection of the femoral head (ball of the hip joint), and the application of an external fixation device.
The groin incision is approximately 1 inch long and would be done in the same place Sam's current scar is. This incision is for the release (tenotomy) of the iliopsoas tendon. This is a very tight tendon that prevents the hip from fully extending in perthes disease. The second soft tissue release is the abductor muscle tenotomy. The abductor muscles are the group of muscles in the inner thigh. This release consists of a stab incision (1/4 inch) on the upper inner thigh. The soft tissue releases allow full mobilization of the hip joint. This in turn allows for the hip to be placed into the proper position and complete containment for external fixation.
After the soft tissue releases have been completed, the small diameter core decompression and bone stem cell graft injection is performed. An arthrogram of the hip is then performed. This is an injection of dye into the hip joint that coats the cartilage and allows for visualization of the femoral head. This is the best technique to truly determine the shape of the femoral head. Also, the hip is taken through a range of motion under live x-ray (fluoroscopy) to allow real time visualization of the hip movements. The arthrogram provides Dr. Standard with information and a starting point before the distraction is performed.
Sam's surgery would include the removal of his existing hardware and the addition of the femoral rod if necessary. After that the hip is placed into full extension and approximately 20 degrees of abduction and the external fixator is applied. This device consists of (in Sam's case) 4 pins above and below the hip joint. The pins are placed through the skin with very small incisions and inserted into the bone.