Couple things I didn't say in the last post…

I was so busy getting the details out for family and friends that I didn’t get to adequately share our gratitude for Dr. Nelson. It’s amazing to have a surgeon who’s an email away at all times. I'm very well aware that the fact we heard back from him in less than twenty four hours isn’t the norm for others. It means so much to have him respond not only so quickly, but more importantly, with such kindness and compassion. 

Having our child undergo nine surgeries hasn’t been easy. It’s been an emotional rollercoaster full of highs, lows and the occasional low low. However, with each procedure we have been confident in Dr. Nelson’s ability and touched by his kindness. It means so much to trust in the person making decisions for your child. As parents we did our best, but there were so many times that we did not know what to do or when to do it. In these moments where we felt overwhelmed or simply didn't know the best decision to make, we leaned on his guidance and knowledge. We knew he had Amaya's best interest at heart. And that was enough. I'm so grateful to have him be a part of our lives for so long. We met him when Amaya had just turned five years old. We were so unsure of the next steps. He was the third pediatric orthopedic surgeon we had met. From the moment he entered the room we were in awe. There was an immediate difference. He was so knowledgeable on her condition and the best way to treat it. He gave us a treatment plan that would start with her first surgery three months later. He gave us an idea of what we would expect for her life and the many procedures she would need. And here we are nearly fifteen years later. About to undergo surgery number ten. And with the same confidence in our surgeon, gratitude for his skills, knowledge and kindness. His presence is such a calming guide as we head into our (hopefully) last procedure. 

Dr.  Nelson and Amaya back in 2012. 

We'll have to make sure to get an updated picture of the two of them 

Surgery Number Ten

Since Amaya‘s last surgery a lot has happened; she’s graduated high school and started college in Pittsburgh, PA. It’s hard to believe we have a nineteen and sixteen year-old in our family but here we are. Each phase of parenting is so different. As they grow they're both becoming more of their own person; each so different and unique and fill in our family with love. Its amazing to look back. We met Dr. Nelson just as Amaya turned five and Elle (Lidie) was two. 

It’s been almost two years since Amaya’s last surgery. During which her Precise lengthening rod was replaced with an intramedullary rod. At the time Dr. Nelson let us know it could stay there or be removed if it was causing discomfort. Amaya had mentioned discomfort during her junior year but opted to not have any surgeries heading into or during her senior year. With the busyness of senior year, graduation and moving out to Pittsburgh the time has gone by quickly. Amaya has continued to mention discomfort but recently has said that it has been harder to deal with. She says the majority of the discomfort is in the areas where the screws are, both near the hip and the knee. She also said the discomfort is primarily when her knee is bent, when her legs are crossed, but also after sitting for long periods of time, after excessive walking, and when she walks on uneven ground.  

This surgery should be pretty straightforward and quick. Dr. Nelson said that any orthopedic near us could do it. Amaya really wanted Dr. Nelson to do it. I think for many reasons; she really trust him, she feels comfortable there as she knows what to expect, he knows her and her leg so well. And if this is to be her last surgery it’s a bit ceremonial to have all ten done with him. We reached out to him last week to see what his availability is for summer. He put the order in quickly and we were in touch with his scheduler two days later. It happened so quickly and in a matter of days we got her surgery scheduled for June 20th. 

We still need to work out the details to figure out but have some time to do that. In the time that we reached out to Dr. Nelson to the time we had the surgery scheduled was pretty quick, just a matter of days, which is great. In those few days I thought and felt so much, as I'm sure Amaya did too. I’m so grateful for Dr. Nelson and his warmth, glad to have it scheduled with some plan in place for Amaya, and it’s exciting. I'm also worried about the logistics and making sure that everyone is taken care of. I'm also hating that Amaya is having discomfort and pain, grateful that there’s something that can lighten a little bit of that. And remembering the past surgeries and procedures and knowing this is the path our family has been on. At times its been challenging; for each of us in our own ways and as a family as well. 

I know for Amaya she was nervous and stressed. Before we heard back from Dr. Nelson and the surgery was scheduled she was nervous about having the surgery and worried that Dr. Nelson wouldn't be able to do it. She worried she would have to get it done by someone else somewhere new. Once we got it scheduled with him there was a sense of relief. Then the reality of a surgery kind of pops in her (our) mind.  

As for the surgery, were assuming it will be outpatient. The last time she had hardware removed it was outpatient. Compared to some of her previous ones this is one of the more "minor" ones. She will have four incisions, the one on the knee being the biggest. 

Thank you for your love and care for us on this journey. 

Amaya making friends with a Lemur in SC when she visited a friend. 

Basketball and Skateboarding

It’s hard to believe that it’s been six months since we got Amaya's last x-rays. Sure enough, it has. In that time Amaya finished her junior year, continued to heal, weaned off pain meds, she's able to walk with minimal pain, she can bend and extend her knee fully (and with no pain). She's been doing really well, so we were anxious and excited to see the new x-rays. We immediately saw a huge difference. I can't explain the amount of joy I felt and the feeling of relief that washed over me. Dr. Nelson was also happy with the x-rays (that only increased our joy and relief!). He said he could remove the rod (as early as July). He also said she could remove it later in life or opt to keep it in. He said its not obligatory to remove it since there were no long term effects to leaving it in. He said its up to individual's preference. So we left it to Amaya to decide.

Amaya had mentioned having some discomfort at one of the screw sites so I figured she would want to get the rod out as soon as possible. She thought it over and decided that she doesn’t want to take it out just yet. The discomfort is minimal and she’d rather have no surgeries heading into her senior year and all of senior year. A whole school year without surgery! She said she wants to leave it there and have a “normal year and get back to playing basketball and skateboarding." She hasn’t been able to do those for several years and she’s really looking forward to getting back to them.

So for now the plan is she gets to be a kid, while she still can, she'll be eighteen in September! She said she'll revisit the idea of rod removal next summer. At that time she'll decide if it's something she'd like to do and if so, when. She may decide to get it removed before moving off to college, decide to just leave it there until it becomes problematic or leave it there permanently. I think we are all looking forward to some “normalcy." To not have to think about the next surgery, prepping for it, the surgery itself and then recovery. 

I can't believe we are at this point; nine surgeries later, two feet on the ground, a stable hip and knee, no pain, no complications. It's amazing! We're so grateful for all that Dr. Nelson has done for Amaya. We met him just before she turned five and here we are a couple months away of her becoming an adult. I’m so happy and grateful she’s doing well. Way back when we had no idea what this journey would be like. I remember googling PFFD when Amaya was just a baby and feeling scared, overwhelmed and lost. We knew little of PFFD and less of the treatment options. We worried what her life would be like, and how we would all manage. It's been wonderful; Amaya has amazed us with her strength, resilience and wisdom. At times its been painful and challenging but we've leaned on one another for strength. We've been lucky to have met and become friends with other PFFD families along the way. Their guidance and empathy has helped tremendously. Our family and friends have shown up for us in ways we didn't even know we needed. We are so fortunate to have an amazing and kind surgeon in charge or Amaya's care. He has treated her with care, kindness and respect. We couldn't have asked for better. Thank you all. We are incredibly grateful. 

Here we all are at Lidie's 8th grade promotion earlier this month. 

Strange to think that a year from now we'll be at Amaya's high school graduation.

Here's the two views of her x-rays. 

Look at that bone! 

on the bottom one you can see the small screw; that's were Amaya feels minimal discomfort 

(usually when she pushes on her thigh).

Good Progress

Well, it’s been about a month and a half now since we’ve been taking care of Amaya’s wounds. I’ve been able to see the healing progress every time we cleaned and changed bandages. For Amaya it was frustrating because it just didn’t quite seem to be going fast enough. The incision on the side finally closed up and scabbed. The top one, which was the biggest, has gotten small enough now that it isn’t needing any Steri-Strips. That’s great news! 

We got follow-up x-rays taken on December 8th, but it took about three weeks to get a copy of the x-rays. The hospital had recently switched to a new system and there was trouble accessing the x-rays and getting a copy to us. It was a bit frustrating waiting three weeks; there were many calls, several visits to the hospital and trying different ways to get the x-rays sent to Dr. Nelson. Thankfully we were able to pick up a disc on the 28th and send them his way. His response brought so much joy and relief. He said, “These x-rays are showing good progress. The bone seems to be significantly more robust and will likely continue in that direction. She can progress to full activity if she has not already. I would recommend waiting 6 months for the next x-ray.”

Wow such a great news! It’s so wonderful to finally feel like we’ve turned a corner and progressing toward healing. 

Thank you all for your love and support

Side view 

X-rays we’re taken two months after her surgery in October (when she got the new rod and her bone graft).

Top wound 

Side wound 

Open wound and lots of stress

Monday night after her shower Amaya called up,  “mom, I want you to see my incision in person.” Ugh, overcome with feelings of stress and worry I headed down. This is what I saw…

I felt sucker punched, I thought things were fine and healing well. In fact, earlier that day one of her teachers  asked me how she was doing. I happily and honestly said she was doing well. She’s off her crutches, her pain is minimal, she’s walking well, and her incisions were healed (or so I thought). So when I saw this incision, one that looked healed weeks and days ago, I was shocked, disappointed and worried. I put some antibiotic cream and a bandaid on it. On close inspection it looked deep, much deeper than I’d like and would expect six and a half weeks post surgery. She also complained of pain at the site, not only when she moved but coming in waves. Worried and stressed I sent her to bed, not how I want to feel before bed. 

I woke up early Tuesday to make some calls. I wasn’t sure if this was an urgent care or emergency room visit. I spoke with a nurse who sent us to the emergency room. The ER doctor said it looks like one of the lower layers had come apart before fully healing so when the top layer of stitches came off the wound opened. He said he was unsure how to proceed; he said it was a tossup between either using a scalpel to open the wound and make fresh edges then stitch the wound or to continue with wound care (daily bandage changes and a lot of waiting and hoping). He asked for my preference which I had none then I asked him for his (after all he’s the doctor). He said if it were him he would go with option one but asked what Amaya would prefer. As the patient and almost adult I felt she should have some input. Through tears she asked that we wait on the recuting and stitching, mostly because of timing. See we were about to start a full days drive to visit family friends. I agreed the timing wasn’t great, but her well-being was most important. I hate the tossups; it’s a gamble and I don’t want to take any extra risks with Amaya’s health. I hated the idea of re-cuting and starting over with a new wound. The doctor said it wouldn’t hurt to wait a week, he suggested adding steri-strips to help gather and close the wound. Then we would continue with wound care and monitor for infection at home. He cleaned the wound thoroughly then applied the steri-strips. It took four steri-strips to close the wound; showing it was bigger than I had realized. He showed me how to replace them and sent me with a kit to do it at home. 

On our way home Amaya apologized for causing us more stress and thanked me for taking her to the doctor. She’s always done that, but I reminded her she’s got nothing to apologize for. This is what we do, we love her and we take care of her. It’s part of the mom job, stress and all. And I wouldn’t have it any other way. 

He added some gauze and tape over the steri-strips so that the wound wouldn’t get bumped on our trip. 

My steri-strip kit…wish me luck. 

 

A month later

Time has been a huge blur since Amaya's surgery. So much so that we went to get Amaya's x-rays this past Friday then realized that it had been three weeks and not four since her surgery. I had counted multiple times and could have sworn it had been four weeks. While we were in the waiting room, Amaya pointed out that I was wrong. We've sent the pictures to Dr. Nelson and are anxiously waiting his reply. Although it maybe too soon to see a difference in bone growth. 

Thankfully she’s doing real well. Amaya said that she’s noticed the area around the broken part in her femur isn’t in discomfort or pain like it was before the surgery, so that’s great news! She's walking without crutches and fully weight bearing. She has almost full range of motion. She has minimal pain that ice and Tylenol alleviate. Walking up and down stairs is what seems to be causing her the most pain. Her wounds are healing well but the stitches are still there and haven't dissolved like we were told they would. I asked Dr. Nelson if there is a chance they aren’t dissolving stitches and we should have her local pediatrician remove them. 

Its been nearly a month now and life is starting to feel normal again. Those first few days after surgery were intense, long, tiring (actually more like exhausting) and draining, especially those first twenty-four hours. Once we got home it was wonderful to be back home and reunited as a family. It took a few weeks to catch up on sleep, and get back on track. We're so grateful that Amaya is recovering well. 

Two weeks after surgery Amaya went to the local pediatrician to have her wounds looked at. She was not feeling the kiddie room as you can see by her face. The nurse practitioner said her wounds looked good. 

Here's a look at her incisions...

they used glue on top of her incision this time which she didn't enjoy how it felt and it irritated her skin

these are the two tops ones where the screws were removed and a new one inserted.

Here's the incision on her knee, this is the longest one and the one that caused the most pain and discomfort. As she walked it was irritating and uncomfortable. That has eased a bit. 

These are on the side just above her knee. 

This was taken three days ago. 

Amaya noticed it was a little pink so we washed it and added antibacterial cream for a couple days.

Here's a look at the x-rays... 

Surgery day

These past two weeks have been a whirlwind, which has been a bit of a blessing as it left us little time to worry. I got in a car accident on the 26th, thankfully no one was seriously hurt, and the girls weren’t in the car. Then busy prepping for this trip and surgery, both at work and home.

We drove to Sacramento stayed with family, then took at 6 am flight to Ontario yesterday. We had breakfast with Paco, Amaya’s grandpa, then did all of our pre-op appointments and duties. 

We’re staying at the Ronald McDonald House and one of the best parts is that one of our PFFD family friends is here. We met Camille when she was 6 months old and Amaya was ten. She’s here for her 5th surgery and Amaya for her ninth. Amaya chose two dolls for her; a Dr. and a girl with a prosthetic leg. So cool those exist now! It was so sweet to see them play and talk. Amaya  got to answer questions and talk with Camille and her parents. I think it helped calmed Amaya the night before surgery, a good distraction and a way to feel like she’s helping. And its so wonderful to know you’re not alone. 

Today we check in at 10:30 am and then have surgery scheduled for 12:30. Amaya’s still resting and feeling good about it. There’s  always two things she dreads, blood work and an IV. We did bloodwork yesterday and this time she choose to watch which she said helped a tiny bit (knowing what was happening). Hoping the IV goes well, last time she ended up with three because they had a hard time getting a good vein. 

We appreciate all the love and good thoughts. Josh and Lidie didn’t come on this trip and were missing them a lot. Thank goodness for texts, calls and FaceTime.

  

Surprise surgery

After the last x-rays (four weeks ago) Dr. Nelson reached out to let us know that Amaya would possibly need a bone graft and/or exchanging of her rod (using the same incisions, they would remove her lengthening rod and put in a nonmagnetic nail a millimeter or two bigger which would help stimulate bone growth). 

We realized he would be in Southern California for a two week window the last week of September and first week of October and that we would be in the area for one of those weeks. We reached out to see if it would be a good idea to go see him. Dr. Nelson said that if she was still in discomfort or pain that it would be smart to go ahead and schedule the rod exchange. He and his surgery scheduler have been working hard for the past two weeks to try to get Amaya on the schedule. 

She's been having discomfort and pain in both her knee and leg. Dr. Nelson had to borrow/steal some operating time from another surgeon. The other surgeon agreed to let him switch if she could observe the surgery since she's never done it. Dr Nelson and his surgery scheduler, Tammie, worked hard to get Amaya seen and we are grateful to both of them for their hard work and dedication. Even though the timing of us being in Southern California didn't match with available surgery dates, they fit her into the schedule.

Even though we weren’t looking forward to an extra surgery (this will be surgery number nine) we are grateful that this will help with healing Amaya's bone and lessen her pain.

At the end of August Dr. Nelson said that if Amaya had an "unexpected amount of healing" we would cancel the surgery. After seeing the x-rays she got this week he said that we'll continue with the surgery as planned.

Here are her newest x-rays, taken on Sept. 13 (four weeks since the last ones). 

It's been awhile...

    I started and stopped this update many times this summer but never quite finished it. I spent summer studying to take and then passed my ASWB exam (woohoo!). I am happy to say I am now an official LCSW.  

    At this point summer has come and gone. We've passed the six month's post-surgery point, which was July 25th. And today marks the six months post lengthening point, Aug.22. We spent summer resting and continuing to work on Amaya's physical therapy homework; she walks a mile a day, does stretches and exercises to build her strength and increase/maintain her flexibility. She had two physical therapy sessions and her therapist has requested more sessions. Once we hear back from insurance then we can schedule more.  

    We've had two new sets of x-rays and updates. Her x-rays are showing that her bone is healing and consolidating. However, the growth is slower than we'd like or expect. The plan in July was to continue with daily walks, continue with bone supplements and continue to monitor the bone growth. Six weeks later we got follow up x-rays and after looking at those Dr. Nelson said the plan now is to have her continue walking, keep an eye on symptoms (pain and discomfort), get x-rays in one month and follow up. If we don't see more bone growth and she's uncomfortable then she may need a bone graft with possible exchange of the nail. He said it sounds worse than it is. They would use the same incision spots as last time and put in a new non-magnetic nail, a millimeter or two larger, which helps stimulate bone growth. Dr. Nelson said, "Probably she'll be up and walking soon after." 

    Amaya is being such a trooper at the thought of another possible surgery. She smiled and said she was frustrated but would do whatever it took to get better. We joked saying she may get to ten surgeries rather than the scheduled nine, and we reminded her that she can get more of the hospital pudding she loves. She was happy about that! We're a bit frustrated but focusing on the positive; she's had a relatively smooth process and only another minor complication when she was six (her bed sore), she's able to walk, she's maintaining her range of motion, and her pain and discomfort is minimal.  

Enjoying the beautiful view after her first walk on an uneven path.

These two x-rays above were taken July 11th. Now compare them to the ones taken six weeks later…

    These x-rays were taken Aug 17th. I can see growth, but mostly on the left side (inside of her thigh) the outside doesn't seem to be growning much at all. Immediately Amaya was disappointed as she noticed a stark difference. She was frustrated ad wondered if the PT was even helping. We tried not to worry until we heard back from Dr. Nelson and tried to stay optimistic. As you can tell there's more growth on the inside part of her femur, especially compared to the outside. 

    Its hard to get a good look at the x-rays because these are photos of screens, so the quality is a bit poor which is a challenge for Dr.Nelson.  

Amaya realizing she could touch her butt with her foot-this was the first time since her surgery in January! 

These two were taken July 25, 2022 on her 6th month anniversary. 

She was excited and even gave me two silly poses. 

First day of 11th grade for Amaya and 8th for Lidie. They're getting so grownup! Amaya was so excited to start school without crutches and to be able to walk around campus. 

Five months

    Today marks five months since Amaya had her lengthening surgery back in January. She's recovering really well and making great strides. Each day and week we see her improving and its great to see. For her it feels like a long journey and at times she gets frustrated. Recently she's said things like, "I just want to be done (with pain and recovery)." And "I want to walk normal." Even though I understand her pain and frustration I have to also continue to help motivate and push her. We make sure she does her daily physical therapy homework, eats well, gets good sleep, stays hydrated and takes supplements and vitamins. These are often contrary to what being a teenager is all about so some days we have our work cut out for us. But her physical therapist reminded her that her body is needing "fuel" to grow and solidify her bone and do her PT homework. 

    The past month Amaya's physical therapist had wanted her to transition from using her crutch to using a walking stick while walking. The next step would be to walk unassisted. Around school she was still using the crutch (more to avoid peers and give them a visual cue to not bump into her or to rush her as she walked). Amaya didn't really need the crutch, but it helped. During her twenty minute walking sessions (part of her PT homework) I noticed that Amaya was reluctant to walk out in public and was having some hesitation about losing the crutch and switching to the walking stick. It had become a true crutch (emotionally and mentally); it was limiting her and preventing her from progressing. We had a couple of talks about it and once she was able to see it for what it had become, a "true crutch" then she began to transition away from it. After three days of using the walking stick Amaya was ready to try to walk unassisted. Now she's happy to be walking unassisted and is getting better each day! 

    When we went to her physical therapy appointment this week she went without a crutch or walking stick. It was interesting because her first thought was, "Will my therapist be upset with me?" (for walking in unassisted) but when she was met with praise and encouragement Amaya realized she was on the right track. This had been her first session in two weeks and the therapist was happy with how well Amaya has done with her homework and recovery. 

    What's next? We will continue with daily PT homework, see the therapist every two weeks with the focus being on working on agility and building strength. We will now get her x-rays every 6-8 weeks and continue to monitor her bone growth. We're giving her a daily bone supplement (with calcium, vitamin D and magnesium) to help with her bone growth. Dr. Nelson wasn't too happy with how her bone growth was at the last x-rays. He's not worried yet but we're keeping an eye on it and he suggested we add the supplements. Amaya's enjoying her summer and resting as much as she can. She's also taking a film class at the local community college and working one day a week. 

At therapy Amaya looked so tall to me! 

She had to go forward, backward and side ways (in both directions) while pulling 60 lbs. 

This is the most weight she's done and it wasn't easy. 

Amaya walking on the bridge by our house for her PT. Not a bad view! 

She's up to 30 min of daily walking which is about a mile roundtrip. 

Stepping and stopping her left leg while holding 20 lbs. 

Amaya did well with this and it was great to see her balance has improved so much! 

Lunges and side steps in the hall while hodgkin the 20 lbs. 

After these Amaya began to tire and asked for a break.

We went outside so Amaya could get a mask break (masks are still required inside the clinic and this was the longest Amaya had worn a mask while doing physical work since school got out) and she had a longer distance to do her side steps. At first she got overheated and had to sit and take a break. She had some water then felt better. 

Her therapist had her try jumping. I gottta say I was very nervous about this and worried she might fall. But she surprised me, as she has many times in her life, and jumped! Then did it again!

New X-rays

  These are Amaya's most recent x-rays. They were taken three months after she stopped lengthening and four months after her surgery. Dr. Nelson said we should continue to get x-rays every 6-8 weeks and continue to monitor the bone growth. He also said for her to continue with her current activity level.

This is the front view of Amaya's femur. Amaya's pt said she thinks there's more bone growth on the left side (inside of her thigh) than on the right side (outside)-I noticed this too. She suggested we mention it to Dr. Nelson. He said it is not worrisome, but that he'll keep an eye on it and monitor the bone formation. 

Here's the side view.

Return of Independence

     One of the challenges Amaya has had during this surgery and recovery has been her loss of independence. It's been mentally and emotionally challenging on her to have to become so dependent on us these past few months. For us it's hard to balance helping her without making her feel powerless and not taking it personal when we're the ones who she takes her frustration out on. Surgeries and the recovery process change the dynamic of our home each time. It's hard (painful, frustrating, and challenging) on all of us not just Amaya. Thankfully, we have a loving and supportive home life and support network.

    This past week we've had some victories and have seen a return of Amaya's independence. Amaya did her own laundry, has been given the green light to drop down to one crutch (for outdoor walking), she tackled stairs at school (this was unplanned since the elevator wasn't working and she had to get up to class. But she did it and was steady and felt safe doing so), and she's started doing more chores around the house. She's also able to do some "small things" like carry her own dinner plate to the table, return dishes, grab a drink, etc. When she had the walker it wasn't safe to do so. These small things are all adding up to be a big thing.

Stairs! She went up and down with no problems! Even though I was terrified the whole time. 

Amaya did her laundry this past weekend! This was the first time since she had her surgery! She was happy, proud and excited! It's great to see her improve and how these victories improve her mental health. 

Side lifts to build muscle, strength and balance.  

During the steps even though we could still see her left leg shake we can definitely tell she's improved.

Her therapist had her practice walking down the hall with one crutch before she would sign off to let her drop down from two to one crutch at school. Amaya is worn out and tired of having to use two crutches so she asked for the change. The therapist hd to make sure that Amaya is using a good gait when she walks. The therapist pointed out that Amaya tends to tilt her hips and turn in her right leg. Once it was pointed out to her she has been working on correcting it. 

Around the house she's been walking without a crutch for about a week now. Although she still uses a crutch to get in and out of the house; we have three steps, with no rail, so that's the safest way.