Fibular Hemimelia (Lydia)

My parents decided to move to California for various reasons, one of which being my medical issues. I’ve got to hand it to them… my parents looked far and wide for answers concerning my condition, what to do about it, and how it would affect my life. All of the surgeons and doctors they saw took one look at my leg.- and its’ very extensive list of issues – and said that the best (and only) choice was to amputate my foot somewhere below the knee. (Keep in mind that this would only solve some of the issues, I would still need surgeries on my hip, knee, and femur) Finally, my parents discovered Dr. Dror Paley; a rising orthopedic surgeon that changed the face of limb lengthening and an expert on F.H. He told us that there was another way and that amputation wasn’t the way to go. My parents had a big decision ahead of them, as I was only two. My first surgery was when I was three years old in 2007. It was a super ankle and an eight-plate installation. It was a year later when I had my first lengthening, which I can explain in more detail. During the operation, they cut the bones in half and put bars into the bones to hold it steady, attaching to an exterior brace. (This is called an external fixator) Every day for three to four months we turn a nob on the ExFix to pull the bone a millimeter apart, and the bone fills in on its own afterwards. Every single F.H case is different. Sometimes it occurs on both legs. In my case, during those four months I had to have physical therapy 5 days a week and let me tell you… It’s no picnic. After that I wait another three-four months to allow the bone to solidify. The lengthening procedure is very difficult and painful, and I have had it 3 times so far. Three times on my tibia and once on my femur. If I am being honest, I’m not entirely sure how many surgeries i’ve had, but it is either 13 or 14. I keep wanting to ask my surgeon each time I have an appointment but I either forget or i’m a little embarrassed that I don’t know. These surgeries include lengthenings, nerve surgeries, knee, hip, ankle, foot surgeries… you get the point.

- Lydia 

Adventures of the Pineapple Room

For a few years, my surgeon, Dr. Paley, lived in Baltimore and that is where we would go for my surgeries. Depending on the procedure, I would stay for somewhere between a week to several months at the Ronald Mc. Donald House. Just the other day my dad showed me a video of Eliza and I as four year olds getting all excited about how we were gonna be living at Mc. Donalds! Sometimes it would be me and one parent, and Eliza and I would be seperated. That was always difficult, especially since we are twins and weren’t used to being apart. Then we’d go back to our house in California. I’m not entirely sure when, but at some point Dr. Paley moved his practice to West Palm Beach, Florida. (near Miami) Deprived of our “Mc Donalds” we would now be staying at a place called “Quantum House”. And so began the adventures of the Pineapple room. Quantum House is a large building where kids and their families could stay while they were undergoing treatment at St. Marys; the hospital nearby. (where Paley now worked) It’s difficult to describe but i’ll go ahead and attempt it. There’s a community lobby and living room that the families share. Down the hall there is a large kitchen/table area where everybody eats. Everybody gets a certain amount of shelf space in the refrigerator and cupboards and… I’m going into too much detail… Anyways, every day a different organization or restaurant volunteers to come in and cook a huge meal for all the families for dinner. Every family gets a room, much like a hotel, with a bathroom, queen bed, and small twin in the corner. They would have fold-up cots available if we needed more beds. It was our home away from home. The dinner system was so great. I loved it because every day would be something different. Mediterranean, american, italian, seafood, etc. Sometimes it would be just pizza or a bowl of chilli, sometimes it would be a big fancy meal made by volunteers. It was one of the best things about Quantum House, especially for Mom. It was oftentimes just us two there and we would be dealing with a lot; physical therapy, surgeries, lengthening, pincare, homeschooling, etc. and it was such a blessing to know that however the day went, we’d always have a hot meal ready for us. Okay… It’s probably time I tell you what the Pineapple room is. At Quantum house, the rooms were labeled with with plants as well as numbers. Our room was the pineapple room. So our walls were yellow, the bed was shaped like a big pineapple, and furniture was tropical themed. Okay relax. I’m joking. A pineapple shaped bed? Really Lydia? The rooms are all the same inside. I guess it made it easier to remember where everybody was as a kid. Avery and her mom were in Mango, Paige and her dad were in Starfruit, and Alex and Luke were in Kiwi. (full disclosure I just made those up… I don’t have that good a memory) Anyways, I remember when I was younger, wheeling myself down the long hall. I would go past each door and imagine that it was like the inside of its’ fruit. I’d wheel past the strawberry room, close my eyes, and imagine the pink walls, fluffy strawberry patterned quilts with beautiful green bed cushions shaped like leaves. I’d imagine that everything smelled like strawberry, and that there’d be a little bowl of them on the desk. I could almost smell the sweet and ever-so-slightly tart scent that would waft by every time the door opened. Of course, it was all in my head. It was always so fun to imagine. Last time we stayed at Quantum house, Eliza and I were twelve. It was just Mom and I for most of it but Eliza, Dad, and even Grandma Phyllis spent some time with us in the Pineapple room. Our days consisted of homeschooling, PT, and goofing off with my new friends. I got my laptop (the same one I’m typing on now) when I was 12 just before we left for Florida. I needed it to continue online school and for something to do in the hospital. It’s a Macbook Air and I remember we had this app called photo booth. It was a camera – video that had all sorts of silly filters and effects. Eliza and I would goof off all the time, making silly videos with weird filters that made us look like aliens. -Lydia

Lydia has graduated from wheelchair to walker to crutches in only two weeks!

This is the easiest lengthening yet!

Reunited

Chrissie and I felt we all needed to be together so Eliza and I flew to Florida for a 2-week visit. Feeling very happy and content. 

Surgery Day

Lydia and Mum before surgery

Sleepy Lydia after surgery. Everything went very well and as planned!!!

Leg Lengthening #3

Well, ready or not, it's time for Lydia's third leg lengthening procedure, so here we are off to West Palm Beach! Chrissie got a bad stomach bug at the last minute so she stayed home and will come in a few days when she's feeling better. We're staying at Quantum House again (yeaaah!)

 Lydia currently has a 6cm lift and that's about as high as she can handle without being too unsteady. As it is, she has a hard time on uneven ground like grass and sometimes trips.

Making friends already in the Quantum House. QH is a residence on the hospital grounds where families of children undergoing treatment can stay. We could not do this without them!!

Breakfast selfie!

Homeschool on Day 1. Dad is pretending to be tough...

Easter Egg hunt with the other kids at Quantum House

Here's a poorly-taken pic of Lydia's leg x-ray

The plan is to lengthen the tibia using the "PRECISE" internal fixator. This is great news because the lengthening rod is inside the bone, so no pins, no pain, no infections, no heavy and bulky contraption hanging onto her leg, more mobility!  (The picture above is an example showing how it fits inside the bone, not Lydia's actual leg). We are very excited as this is the first time we'll have had this type.

Born "Different" (Lydia)

My name is Lydia Schroeder. I am now sixteen years old. I believe I was twelve when I started this blog and haven’t been back to it for years so let me catch you up. I have a twin sister named Eliza and two older brothers. When I was born, I had a lot of problems with my left leg. Fibular Hemimelia. For those of you who don’t know, “Fibular Hemimelia is a birth defect where part or all of the fibular bone is missing, as well as associated limb length discrepancy, foot deformities, and knee deformities. Fibular hemimelia (FH) is a very rare disorder, occurring in only 1 in 40,000 births.” that’s the definition, however, no FH patient is the same. In my specific case, I was born with no fibular at all, only four toes, a deformed knee, hip, and ancle, leg length discrepancies, a very slowly growing leg, and a twisted foot. All of this occured in my left leg. My identical twin, Eliza, doesn’t have any leg issues. This is my story. I was born in England with my identical twin sister, Eliza. My parents decided to move to California for various reasons, including my medical issues. I’ve got to hand it to them… my parents looked far and wide for answers concerning my condition, what to do about it, and how it would affect my life. All of the surgeons and doctors they saw took one look at my leg.- and its’ very extensive list of issues – and said that the best and only choice was to amputate my foot somewhere below the knee. (Keeping in mind that this would only solve some of the issues, I would still need surgeries on my hip, knee, and femur) Finally, my parents discovered Dr. Dror Paley; a rising orthopedic surgeon that changed the face of limb lengthening and an expert on FH. He told us that there was another way and that amputation wasn’t the way to go. My first surgery (I believe) was when I was three years old in 2007. It was a super ankle and an eight-plate installation. It was a year later when I had my first lengthening, but I can explain that in more detail later. (see my Fibular Hemimelia page) January 19, 2017

Mom's turn in Hospital! June 2013

Shortly after Lydia's surgery, my symptoms returned and worsened. I had very little energy, my weight went down to 106lbs, I was weak, nauseous, can hardly walk,  can't stand up straight, my vision is blurred, my blood  pressure read 69/40 and my sodium level was at a critical level of 112 (suppose to be between 135-145). I shouldn't have survived. When the blood test was read (over a week after it was taken(there's another story there)) the GI doctor who was my only doctor at the time, told us that I needed to be admitted straight to hospital. I was intravenously fed with saline solution for 6 days while receiving tests from kidney doctors followed by an endocrinologist. On day 6, we had the diagnosis- Addison's disease and Hashimoto thyroiditis- both autoimmune diseases. And on day 6, I was a new woman! Hobbling in to hospital and dancing and laughing out of hospital. And after a year of my health deteriorating slowly, what a relief to have a diagnosis and treatment plan. Again, I thank Jesus that we decided not to lengthen Lydia's leg in March/April as it would have come upon me in the 4 month period in Florida and the story may have ended quite differently.

Foot Fusion- and Unexpected Hip Surgery- APRIL 2013

From a changed date, last minute cancelled flights and the costs of a damaged mercedes benz we lost quite a bit, financially. So to keep further costs as low as possible I was to go to Florida with Lydia and Tim was to stay in CA. This in itself was a nerve racking decision because unbeknownst to us, I was undiagnosed and suffering from Addison's disease and Hashimoto thyroiditis. My symptoms had left me with very little energy and strength and I was nauseous most of the time. But, I thank God that, for a few weeks my symptoms were lifted and I was able to go to Florida.
Foot Fusion- This is not a surgery Dr Paley chooses lightly. He will only do this if there is no range of motion lost in the process. Lydia had no range of motion in this joint because of her anatomy and the deformity her condition created. Pre-op done, papers signed, prayers sent, Lydia goes into surgery the next next morning. She always blows me away with her calmness before they wheel her into the operating room and I find it very contagious. I was so calm, in fact, that I went back to Quantum House to do my daily task of vacuuming the hall (shocking when I look back), then I went back to my room and prayed a few confident prayers.  A couple of hours later I headed back to the hospital. Now I happened to be reading "Kneeling We Triumph" which I will come back to later. Dr Paley came out into the waiting room and told me that the foot fusion was a success but when they removed a suture in the hip/leg joint area the leg came out of joint and they were going to have to do a hip osteotomy. They required me to sign new papers giving permission to perform the surgery. A side note to this is that Lydia had often complained about her leg feeling like it was popping out of it's socket. I have been so used to her complaints that I have been guilty of switching them off- I guess that's the wrong thing to do. So there I am in the hospital waiting room feeling very much more anxious that Lydia may experience the same nerve damage issues as she did before when she had the other hip osteotomy. I sent texts to my christian sisters to pray for her and just about held it together because of my faith. Then the big blow came. Another doctor, I think it was Servando, came out to see me. They couldn't get her leg to go back in position because of her tight muscles and so they needed to do a Femoral Shortening and needed me to sign papers to allow them to do this. I was devastated. Taking away bone length from a bone Lydia had previously and painstakingly lengthened earlier was such a blow. My heart was broken for her and my calmness gone. I signed the new papers.
It just so happened that I had read in my book on prayer, only a few minutes before Servando came out, about honesty in prayer, God knows our heart after all. So this read, I got real real honest. My prayer changed from- please give us the strength to deal with this extra surgery etc to- Lord, this is too much, I don't want this for my daughter, you are pushing me too far.
Waiting…waiting hours later, Dr Paley came out. He was very happy with the Hip osteotomy and had a lot of confidence in the new joint and …..they didn't need to do a femoral reduction!!!!! Tears of sadness turned to tears of gratitude and joy and strengthened faith! And oh, what a blessing indeed that we did the U-turn in the first place and stopped ourselves from doing the lengthening at this time! And what a blessing too that the suture held her leg in place up until she was on the operating table when there was so much pressure on it with the tight muscle.
Tim managed to fly out when we realized just how much more Lydia had to deal with and all the extra lifting. It was very necessary especially with the journey home! Thanks Tim!

Here we go again...

Well it's decided then. As much as we don't want to lengthen now, we don't want to put her through TWO surgeries. It breaks my heart to think how long she'll be out of action and that she'll miss an entire summer, but it has to be done sometime, and it's better to get it all over with now.  This will be the last lengthening of her tibia ever. So we're going ahead. Surgery is booked, airline tickets are bought, time to start packing and make the best of it!!!

Changed minds. March 2013

About this time last year, Lydia was due to have a foot fusion and second lengthening combined. However, big doubts about the outcome began to invade our peace of mind. We had a surgery date, booked flights, cases packed  and everything planned. Only 1 day before our flight, and after a few days of prayer (me) and talking it out, we decided to cancel the trip and opt for the amputation and prosthetic. Now, because of the stress of a changed plan and a irreversible plan at that, the four of us- Tim, Eliza, Lydia and me, took off up the coast in an RV to recover.
Why did we choose to amputate? Well, we were disheartened by all the pain issues Lydia was having so often. I would walk the short distance to school with the girls and Lydia would stop half way and refuse to go on because of pain. The school nurse would call me frequently with reports of Lydia's knee/hip/foot pain… It was not the life we had in mind for our little girl and we thought she'd have a better quality of life with a prosthetic.
Watching the girls play on the beach on the back of this decision was heart breaking. I couldn't stop staring at Lydia paddling in the water with her bare feet and just stared at her from a distance with tears streaming and a heavy heavy heart. To ease my nerves I began watching informational videos about how prosthetics are fitted… that made my heart heavier still and I was filled with regret. How strange it is, when you so confidently make a decision, feeling so at peace with the new road ahead and then, WHAM, full regrets fill your very being.
On the way to our beach break we backed into a Mercedes with the RV. And on the journey home, the Janis Joplin song about Mercedes played on the radio. Which I would never have included in this post only it triggered my imagination and resulted in my little song about this very crazy time in our lives.
I am very thankful Dr Paley found out about what we were planning to do which resulted in him calling us. Such a busy, well known and highly respected doctor may have just written us off- but he didn't and when we were home we had a conference call with him. He was patient, understanding and reassuring and after hearing what we had to say, suggested that a foot/ankle fusion without the lengthening until later so that we could be confident and reassured that Lydia would be pain free, would be the best option. Our U-turn, U-turned!! We came full circle and ,oh, what an emotional ride it was.

Two years later....

Two years without a new post!  I may have to go back and enter a few retro-actively...

Things have been great for Lydia over the past two years. No surgeries, and she's done really well, until about 6 months ago when she started experiencing pain in her foot and sometimes in her hip too.  Some days it's OK, other days she comes home from school early due to the pain, hopping on one foot, using a cane, and sometimes even a wheelchair. So as I speak we are in Florida to see what Dr. Paley thinks we need to do to alleviate the pain.

After looking at her and a fresh set of x-rays, Dr. Paley wants to re-position her foot to point in a more normal position. Currently it points downwards a bit, and can't bend more than 90 degrees in relation to her leg. This means she can't walk with her weight squarely on her foot, all her weight is going on the pad and none on the heel.  This entails cutting the tibia at it's lowest point and angling it up a bit.

He said we can do this in conjunction with her second and final tibia lengthening, or separately.  Doing it together would mean one surgery not two, but we're not at all prepared for a 7-month lengthening so we will have to think it through carefully.  He's also concerned about her hip, and has ordered a catscan to further evaluate it.

I am torn between relief knowing another surgery is not imminent, and trepidation about the one that is surely coming before too long. It's so hard -- harder than I thought it was going to be, but I won't dwell on this as that is a slippery slope that I have been down before and this is not the place for it.

Lydia after a 3 mile hike

OK it's about time for some good news!  Lydia has really shined this year and pulled through the difficult recovery. So much so that we went on a 3-mile hike in Santa Barbara today!  I am so proud of her -- nothing seems to slow her down.  Tim.

Ankle surgery to add an "8-plate"

Although Lydia has been doing very, very well, it's time for another minor surgery.  After the second ankle surgery, her foot was much more stable however it has a slight downward point, so that it's as if she's walking around on her tiptoes all the time.  Luckily it's not creating any problem for her, she's walking well and not in any discomfort.  But Dr. Paley wants to add an "8-plate". It requires a small incision and they insert a small plate and two screws to tether the growth plate of the lower tibia and guide it to grow the foot out of its dropped down position. No bone cutting, no cast, she can resume weight bearing right away. It is an outpatient procedure. Over the next 1-2 years this small plate will encourage her foot to turn upwards as she grows.  As always, I hate having to do this, but this is the path we chose and we need to stick with it and be strong for her sake.

Ankle pain is gone

Well, as much as we hated to have to go back again, it seems it helped a lot.  Dr. Paley saw where the nerve had been pinched and he "decompressed" it.  The pain went away almost immediately, so that is something to be very happy about. Lydia is doing much better.  She still has little movement in her foot though.  Dr. Paley said it can take a long time to recover, and that they usually do, but I am somewhat skeptical.  I believe her nerve is damaged and will never completely recover.  Hopefully it will recover enough to not be a problem.  We knew from the beginning from Paley and other parents that there are always some complications in these processes, and I guess this is hers. Given the alternative of amputation, maybe this isn't so bad :)