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.

Community

It is a true gift to have people who get you. Its been nearly a week since we scheduled Amaya's surgery and I've already started to hear from our PFFD friends. These are parents who also have a child with PFFD. They've offered kindness, empathy and advice. I have a phone called scheduled with a PFFD mom this weekend to chat about Amaya's upcoming surgery. Her son, who's pretty close in age to Amaya, underwent lengthening with the Precise nail last year. She's already helped me so much! I can't wait for the phone call where I can ask all the things that I've been curious about! I've anxiously been searching the internet like crazy hoping to find any information. It'll be great talking with someone who's experienced it first hand.She shared some of the things we can expect for Amaya...

• that'll shell spend a few days in the hospital recovering

• that she'll use crutches (for quite awhile)

• that she won't be able to bear weight fully for awhile

• It'll be tough, but much easier than the external fixator (yay!)

We also heard from other families across the country offering support via zoom visits, and well wishes. Hopefully we'll be seeing another PFFD family from Oregon on their drive through Northern California late next week.

In addition to our PFFD family we are so lucky to be surrounded by amazing family and friends. Our community is amazing! These are the people who when they tell us, "we got you" I know that they do. Already the offers have come in to help care for Lidie, to feed Cocoa (our cat), make us dinners, let us vent, pick us up from the airport, research and help book our flights, and so much more.  

I'm blown away by all the love and support and I am so grateful for each of you. Thank you

When we got home today we had a package waiting for us. Our sweet friends the Wasserman's sent this for "Amaya the Amazing! The Crutch Buddies give padding to make using crutches a bit more bearable. These are the ones they used for their son and recommended them for Amaya. What a lovely surprise! 

We've got a surgery date

Friday morning I got a voicemail letting me know that Amaya's surgery had been scheduled for January 25, 2022. I was instantly happy, nervous and relieved. A minute later I was hit with a wave of emotion. I sat and cried. As happy as I am that Amaya will get her leg issues corrected and pain relieved I couldn't help but think, 'they're going to break my baby's leg.' Mostly I hate that Amaya has to deal with ongoing pain. I hate that she has to deal with surgeries and painful procedures. This will be her eighth surgery, eighth. 

At times I get sad and frustrated that this is Amaya's life, our life. And then the moment passes and gratitude washes over me. I'm grateful Amaya has an amazing surgeon. I'm grateful she has a treatment plan and that there's an end in sight for her pain. I'm grateful to have such a loving and supportive community. 

I got to spend lunch with Amaya, one of the many perks of working in the district where she attends. As soon as I told her her hands began to shake. The first thing she said was, "What?! You just say that out of nowhere?!" She was filled with all sorts of emotions. But mostly she was glad that a date was scheduled. The more she thought about it she began to worry about the thing she dreads the most...getting an IV. I suggested we not worry about that yet. And she agreed. So we focused on the good stuff...doing something to get rid of her pain.  

As the day and weekend went on Amaya began to ask questions...will she have to use a wheelchair? or a walker? If so, for how long? How are we getting down there? There are still so many details to figure out...traveling down, prepping for the surgery, taking time off work, etc. I'm beginning to prepare, make plans and arrangements and begin to research what's to come. Once that gets to be too much I take a moment to just breathe and to reach out for help and support. 

As we plan and prepare over the next two months the thing that will keep me going is our community. Made up of family, friends, coworkers and our PFFD families around the world. Thank you all.

Answers & a plan

    It's been three years since we last saw Dr. Nelson. Amaya was a 5'3 1/2"thirteen year old getting ready to have her eight plate removed. Even though I was taller than her, she seemed so tall and grown up. She's grown so much in these short years. Each time I look up at her I'm reminded of that. 

    At that last visit we made plans to come back for a follow-up once Amaya had finished growing (Dr. Nelson said that would be at about age 14). He projected she would grow to be 5'8." She now stands at 5'8 & a 1/4" I'd say that's pretty close (amazingly close!). He had made additional projections and gave possible treatment options but we were in a "wait and see" point in Amaya's journey. 

    The past three years she's grown but unfortunately her knee pain has also grown. Amaya began to mention she had knee pain a couple years ago. It's hard to remember exactly when because at this point it's become the norm. She's got a high tolerance for pain and don't mention it often. At times we could pin point and explain the cause; a busy day, lots of walking, playing basketball, etc. Over the last year she began to complain of knee pain more often and at times for no obvious reason. Sometimes she would wake up in pain, or if she moved her leg a certain way, or just laying in bed. The last year or so we've have a hard time getting her any pain relief. We tried a customized knee brace, ibuprofen, rest, massages, muscle relaxing creams, chiropractic treatment and created a 504 to give Amaya much needed accommodations at school. All gave minimal and momentary relief. 

    I've been calling to make an appointment to see Dr. Nelson for over a year. The Covid pandemic made that extra challenging. Between quarantine restrictions we were placed on waiting lists and continued to call. In October  I got a call that there was a last minute opening and we could go down the next week if we wanted to. To be honest I immediately panicked. I didn't think I could make the work & travel arrangements in such a short time. Thankfully friends reminded me to go for it and that things would work out, which they did. When I called back Monday we were able to secure the appointment and then we had two days before we left. It was a whirlwind. 

    We left Wednesday morning and began the ten hour drive down to Southern California. The morning of Amaya and I were both nervous, anxious and full of emotions. We were both hopeful there would be an explantaion for the pain and a way to resolve it. The appointment was wonderful! It was so great to see Dr. Nelson and to be have him treat Amaya. I instantly felt calm and was confident that she was in good hands. He reviewed x-rays, watched her walked and then manipulated her knee. He said that overall she's doing great! Her gait is good, her knee is doing well overall as well (especially for a CFP knee). He said it made sense that she was in pain and there were a several things he could correct. 

So here's the plan...
Phase One...Dr. Nelson will release the scar tissue on the outside of her thigh that was left there from her external fixator. He will also perform a femoral osteotomy, to correct the misalignment in her femur. Lastly, he will insert the Precise nail to do a femoral lengthening. She will undergo femoral lengthening to gain 2 cm length to compensate for her discrepancy (this will take about 2 1/2 weeks). Also we need to get an MRI to evaluate the cartilage as well as the TT-TG (to measure the patellar translation). 

Phase Two...a year after the lengthening her Precise nail will be removed and tissue will be placed to help hold her kneecap in place. 

    It was great to get answers and to know something could be done. Amaya was so grateful for that! Dr. Nelson gave her the option to wait until summer to begin phase one or to do it at his first availability (December or January. Without hesitation she said she'd like the soonest appointment. She explained that for her its a matter of how long she wants to stay in pain. She's ready for the pain to be over. And we're ready for that as well. Its heartbreaking as a parent to watch your chid suffer and be powerless to help.  

I'm so grateful we got to see Dr. Nelson, to gert some answers and a plan and to have been able to sneak in visits with family members. 

It's hard to tell in the picture but she's rolling her eyes and said, "ugh, do you have to?" 

The answer was yes, yes I have to. 

Here's a look at Amayas x-ray.

We snuck in a visit to Rhino records where Amaya picked up a Bikini Kill record.