Limb Lengthening

Its pretty amazing that you can lengthen a bone. Actually you don't lengthen it, you grow it. Amaya grew  new bone. When she was six years old she grew 3.2 inches of new bone and soft tissues. It was an amazing experience! At times painful but also filled with moments of strength. It is amazing to write that, 3.2 inches! 

If you're not sure how limb lengthening works here's a bit of what I know. Typically in other surgeries they do the procedure to correct, add or remove something then within a few days or so you being to recover. Lengthening is different in that it's a process. The initial surgery is to break the bones that will be lengthened and to insert the device and then the lengthening process begins. It takes time to complete the lengthening and to heal from it.

There are three phases to lengthening latency, distraction and consolidation.

Latency phase...there is a pause in the process to allow for rest. This happens once the lengthening device is inserted and lasts about 5-7 days prior to beginning the lengthening process.

Distraction Phase...this is the gradual process of pulling apart the bone. New bone (regenerate bone) is created in the space. The bones are pulled apart slowly (about 1 mm per day usually done in three or four times throughout the day). As the bones are continually pulled apart new bone is created, and soft tissues grow (blood vessels, skin, muscles, nerves, etc). Frequent x-rays are important during this phase so that bone isn't created too slowly (if so then the bone can begin to harden prematurely) or too fast (this can lead to unhealthy bone). Patients must have regular physical therapy to help grow soft tissues and to ensure that range of motion isn't lost. 

Consolidation Phase...during this phase the new bone begins to harden and calcify. This can take about twice as long as the distraction phase did. Weight bearing is usually increased during this time to encourage healing. The lengthening device is still worn during this time. 

Last time Amaya had an external fixator to lengthen her bone. She grew 3.2 inches, had physical therapy nearly every day and she wore her fixate for seven months. Below is an x-ray of Amaya's femur & fixator from last time. 

This time Dr. Nelson will be doing a lengthening using the Precice nail technique. Rather than an external fixator she will have an internal rod. This should be much easier and less painful. The internal device is much more comfortable has no pin sites (basically open wounds we had to clean and dress daily. This was the greatest source of pain for Amaya last time). This time she is only growing 2 cm (about .78 inches) so that is a much less time to lengthen. We can lengthen 1 mm per day so that's 20 days to lengthen. Then it should be about another month or so to consolidate her new bone. She will wear the device for about a year and then will have it removed next year (and he will work on her knee then). 

Dr. Nelson has created a video showing how the Precice nail is inserted and he talks about how it works. The video has a clip of a real surgery so if you're a bit squeamish you might want to skip through that part (3:45 to 8:40). I'll be honest it was much easier for me to watch this prior to having Amaya's surgery scheduled, now that it is I can't bring myself to watch it. Back then I found this informative, helpful and interesting. Now it makes me squeamish and emotional so I've stopped watching it. I'm glad I watched it before so I know what will be happening and how he will do it. 

If you'd rather skip the video here's some photos that shows what it looks like. 

All these pictures are from the Int'l center for limb lengthening at Mt. Sinai and can be viewed at limblength.org. They also have other videos and information that are pretty helpful especially if you're going to be starting a lengthening soon.

Less than a month to go

Yesterday Amaya let me know that we are less than a month away from her surgery. I just looked and today is exactly four weeks away from her scheduled surgery date. I had been so focused on trying to get her MRI scheduled that I lost track of time. I think thats a pretty good thing. Amaya said she wants get it over with and feels ready. That's huge! I think having a good mindset heading into surgery and into a lengthening process is so important. 

As for me I'm feeling good, for now. I'm prepared (mostly), optimistic and ready to get her some relief. I've secured care for Lidie and Cocoa (our chubby cat), and made travel arrangements. That was the most important thing to do. I've got checkmarks next to most of my to-do list for the trip. I've received help and advice from so many and will continue to do so. That is a wonderful feeling.

I'll be honest it hasn't been completely easy. Around November I was all kinds of emotional. I felt many things; sad, worried, frustrated but mostly overwhelmed. Not knowing what was to come. Even though it's similar, it's different in many ways. Its new and unknown; and it is very hard to prepare for the unknown. Where do you even begin? I was searching for what to expect and found many short videos and a few pages with info. Lots of bits and pieces of information but nothing comprehensive or tangible. Thankfully another PFFD mama reached out and we had a phone call that answered many of my questions and helped me know what's to come. Her son is close to Amaya's age and he had his Precise lengthening about a year ago. Her information as invaluable. 

Toward the end of the month Josh and I realized we were staying close to home and kind of in our own little bubble. It felt like we were "cocooning." We were doing nothing but trying to just get by. Managing life; work and school, and kids, and all of life is challenging then adding this extra thing felt like too much. We were managing but anything "extra" or that felt extra, even the good and fun stuff, felt like too much. We cocooned ourselves to protect ourselves. The problem with any barrier is that it keeps everything out. Even if it was meant to keep the tough stuff out it also keeps the good stuff out. We weren't venting or sharing or reaching out or allowing friends to take care of us. 

After allowing myself time to feel my feelings and making time to stop, decompress and recover then I was able to reach out to friends, and move on. Friends are wonderful! How they can love on you, help you and support you in a non-judgmental way is amazing! I also decided to live in the moment and practice mindfulness to help manage myself stress. They both helped and have helped me get through the MRI nightmare. 

So with less than a month to go my plan is to continue to take care of myself and our family, reach out as needed, finish the last minute preparations and keep up with self-care.

MRI frustration

Part of Amaya's treatment plan was to get an MRI of her left knee. Dr.Nelson wrote he wanted to "evaluate the TTTG and evaluate knee cartilage." I had no idea what it meant when they said, "evaluate the TTTG" so I had to look it up. TT-TG stands for tibial tuberosity-trochlear groove distance. One of the things they look for is to see if there's patella joint instability and how much. Dr. Nelson wanted to have this done so he would know how to treat her knee pain especially after her internal fixator is removed (a year after it is installed, so sometime in January 2023).

The MRI was ordered on October 14th  and we didn't get it until December 21st. Over ten weeks! Ah! Just typing that is unbelievable! The process was incredibly frustrating, time consuming, exhausting, emotional  and at times out right overwhelming. I ended several calls in tears, sometimes out of frustration, sometimes out of anger. 

I wanted to update on the process during that time while I waited but I didn't want to type it until the MRI was scheduled or done. I felt that typing it while waiting was like giving up or just complaining. I wanted so much to be able to post good news. I had no idea it would take that long to get the order to be transferred, to be made an external transfer, to get insurance approval, to get the order and insurance approval to be made an external order, and then scheduled. Even now I don't fully understand the process. 

I expected some bumps since we got the order at her children's hospital in Southern California and we live in Northern California. But I was hopeful since we pay for the higher PPO plan. I could not have imagined that it would be so difficult and challenging. I felt like I was jumping hoops and as I did new hoops were added, moved away and then set on fire. 

Knowing Amaya needed it, knowing we had a time crunch (she can't get an MRI when she has her internal fixator implanted, so we had until January), and knowing she was in pain added to my stress. It is painful to know your child is in pain, especially frequent pain. There are few things I can do to actually help alleviate her pain. And when there is something that I can do to help her I try my hardest to do it. When I'm met with obstacles and resistance well that makes no sense and is incredibly frustrating. 

Along the journey I made several friends who helped me. People who were just "doing their job" not realizing that they were also alleviating my stress, helping me carry this burden and advocating for me and Amaya. I am incredibly grateful to them all. I'm also grateful for sweet Amaya who would often thank me for my efforts and encourage me. I shared updates with her but tried to not let her know how painful and overwhelming it was. Trying to find the balance between informing her without giving her more to worry about. Thankfully I have a wonderful crew of friends (TNC) I can rely on to vent to, who were frustrated on my behalf, who let me cry and share and kept me going. Thank you all.

To all the other parents out there trying to deal with multiple locations and insurance companies, waiting for approvals and fighting for appeals I send my deepest empathy and love to you. It's a tough road we are on. Feel free to reach out if you need to vent.