Thursday, April 23, 2015

Soon to be an amputee... (Fibular Hemimelia / Postaxial hypoplasia)

Preface: When we found out Siena had Fibular Hemimelia / Postaxial Hypoplasia we had a hard time finding information about it and decided to try and document her journey. We hope this will help others who have to make this decision for their family.

The Background

Almost 4 years ago, our daughter Siena was born with a condition called Fibular Hemimelia (more recently named Postaxial Hypoplasia). She was also born with 4 toes on that foot (her right) and a club foot.

When she was a baby she had her Achilles tendon clipped (Ponsetti method of correcting a club foot) and wore multiple casts over 6 weeks to straighten and stretch out her foot. Once the correcting casts were done, she wore shoe braces (Dobb's bar) at night for the next 2.5 years.

She then resumed to "normal" life. We visited doctors in Utah while we were going to college about her condition and were never really told that she had Fibular Hemimelia or great detail on what may be ahead for us and for her. At the time, we were basically told that she would eventually have a limb lengthening to correct the length discrepancy in her right leg. We then moved to Austin, Texas for work and we began to go to the Scottish Rite hospital in Dallas, Texas.

Mommy and Siena at the Scottish Rite Hospital in Dallas

That's when we were officially told that Siena had Fibular Hemimelia and the doctor told us to go research it. That's when we first realized that amputation was a pretty common procedure for this condition. We were pretty taken back at first at the idea of ever doing that. In the first few years of life, Siena was always told that she had a relatively "minor" case and that limb lengthening was most likely the correct course of action. It wasn't until we moved back to Utah recently that we began seeing yet another new doctor that we were told that amputation might actually be a good option for us. We were again pretty shocked as we had been led to believe that limb lengthening was the way to go. At this appointment we were also told that Siena had to be casted again since her achilles tendon had tightened due to walking on her tippy toes. After the casting, she was told that she had to wear ponsetti braces at night to prevent this from happening again.

Siena getting her new cast at Primary Childrens
Siena getting her cast taken off at Shriners
We have since spent the last 2-3 months researching, reading blogs, talking to people who have gone through both procedures, talking to many doctors, consulting with family, praying, pondering and trying to make the best decision for Siena. We heard about the Shriner's Hospital in Utah when talking to another family who had a double amputee and headed there to get a second opinion (or third or fourth...). In that first meeting we were still pretty conflicted on which path to take even though we had done a lot of research. When you do research on the internet everything is very biased one way or the other and most people you talk to are very biased as well. This makes it tough to make your own decision. At the appointment we were leaning towards amputation but weren't sure yet and weren't very vocal to the doctor. After analyzing Siena and some old X-Rays that we brought with us from Texas, we were again told that we should keep heading down the path of limb lengthening. We went home confused and did more research about limb lengthening. We looked into potentially going to Florida or Maryland to visit the leading experts in limb lengthening procedures (Dr. Dror Paley in Florida or Dr. Standard/Dr. Herzenberg in Maryland). This would require us temporarily relocating to the location for a few months to have the surgeries and some physical therapy there. The more we continued to look into limb lengthening, the more we continued to feel that it wasn't the right way for us to proceed for Siena. We then set up another Dr. appt at Shriner's hospital with a new Dr. In this appointment, new X-rays were performed and we learned a lot of new information that we didn't know about Siena's case. We will attempt to summarize this below and we will talk about the treatment options later.


 Here is a list of everything we currently know of:

1. Shorter tibia and fibula (approx. 2-2.5cm.)
2. Shorter femur (approx. 1.5cm.)
3. Under developed fibula - a normal fibula would be longer than the tibia but her's comes to the tibia
4. No ACL
5. Knock knee (Genu Valgum)
6. Ball and socket ankle
7. 4 toes (extremely cute 4 toes)
8. Minor Hip Dysplasia
9. Valgus Ankle / Club foot (tight achilles tendon and stiffness of the ankle)

X-rays from our appt on tuesday

Before we go into our decision here is some info on the pros and cons of either path and a lot of the information that we considered as we have pondered this.
THE OPTIONS (Caveat: we are a little biased at this point now that we have decided and by no means do we think that anyone who doesn't agree with us is making the wrong decision. It's a very personal choice and we would never judge anyone regardless of their decision...)

There are essentially 2 options. 1) Do the amputation in the next several months or 2) plan on doing reconstructive surgeries (including limb lengthening) throughout her childhood. We wanted to write out what we view to be the pros and cons of each. This has been a matter of much concern to us and we have definitely done our fair share of research and due diligence and hope that this might be a way for others to understand our decision and to help other people in the future make their own decision. We will attempt to be as unbiased as possible although we have come to our own conclusion that works for our family. We are aware that many people have strong opinions about this subject and we know that whatever decision we make, there will be hard times ahead and hope our friends and family will be here to support us.

We are obviously not doctors but here is our synopsis of both options as we have understood it...

Option 1: Amputation 

Overview: Siena would receive a Syme's amputation which is an amputation of the foot (it would be right below the ankle joint and she would keep all the cartilage in the stump). The heel pad would then be used to give a sturdy weight bearing stump. Usually this is done around 10-12 months but Siena would have it done around 4 years old (in approx 2 months).

  • Siena can move on and begin adjusting to her new life as an amputee. Studies have shown that if you do the amputation early in life, you are less likely to experience "phantom pain." Also she most likely won't remember the surgery or what it was like with both feet.
  • Fairly predictable outcome - Minimal complications and risks - you know what you are going to get with an amputation - less uncertainty
  • Less residual surgeries (residual surgeries will most likely only involve the knee / femur and be fairly non-invasive)
  • Complete mobility and function of the leg and is comparable to that of a normal leg.
  • Ability to walk, run, swim, skate, etc.
  • Only one major surgery and Siena can go about her normal life (a minority of children with complicated cases may need more surgery down the road)
  • With amputation we would avoid surgeries of the ankle, lengthening of the fibula (approx. 1 - 1.5 year process of surgery, recovery and therapy), lengthening of the femur (approx. 1 - 1.5 year process of surgery, recovery and therapy), and hip surgery (most likely)
  • Siena will not have her own foot - potential for self image issues
  • She would need different legs for swimming, heavy running, etc.
  • Having to deal with stares, questions, etc. that accompany being different.
  • Each prosthesis requires her to re-adjust how she walks
  • We will have to clean out the prosthesis every night and let it dry overnight
  • Depending on the prosthesis, you might not be able wear it at the beach or it may require heavy cleaning afterwards (and same for other dirty outdoor activities)
  • Cost is high for prostheses (most cost should be covered through Shriner's until she is 21)

Option 2: Limb Lengthening and reconstructive surgeries

Overview: The doctor basically explained that Siena would have several reconstructive surgeries throughout her childhood and teenage years. These include surgery of the foot and ankle (potentially multiple times), lengthening of the fibula (1-1.5 year process which is very painful and intense), lengthening of the femur (1-1.5 year process as well), knee surgery to correct knock knee, hip surgery to correct the hip dysplaia. A good chunk of her childhood and teenage years would be filled with surgery and recovery. And the end result would likely be a stiff ankle that is partially functional and a leg which may or may not be fully functional. There is always the chance that you will have to amputate down the line as well if more complications arise. Due to the fact that she has so many issues in her leg, the likelihood for complications is higher.

  • Siena keeps their own foot
  • She may end up with close to normal function of the leg once she is an adult.
  • We don't have to make the difficult decision for Siena to amputate before she is old enough to fully participate in the decision-making process.
  •  Several surgeries must occur over several years ranging from ankle surgeries, leg lengthening surgeries, club foot corrections, knee surgeries, hip surgeries etc. And there is a chance that she will continue to have surgeries her whole life to keep correcting her ankle or other joints/limbs.
  • To do the leg lengthening the doctor would go in and cut the bones and attach a large external fixator that by using pins would be drilled into the bone and help to stabilize the leg while the cut is in the bone. Over about a 2-3 month period of time you literally crank the machine to move the bone farther and farther apart until the leg is at the desired length. After the desired length it met, Siena would continue to wear the external fixator for about another 2-3 months  and then a full leg cast for another few months to help that bone continue to solidify. After this she would have months and months of physical therapy to try and regain motion in ankle and knee as well as adjust to normal life again. If you want a specialist to do all of this (which is smart) you would have to go to Florida or Maryland for months or do lots of travel. Siena would undergo hours of intense physical therapy during the long rehabilitation process. 
  • If they aren't able to get all of the length corrected (you can usually only lengthen 20% of the length of the bone in one lengthening), you may have to do this multiple times. Many people end up with infections in the pin sites which are extremely painful and can complicate the whole process.
  • The doctor told us that by lengthening the limb, you will severely damage the cartilage on either side of the bone and Siena would be left with a 30 year old ankle / knee / hip. We would also have to do hip surgery due to the pressure the lengthening would apply to the hip.

  • Siena would likely be left with pretty significant scars on her leg and a leg that is much skinnier than her other leg. The foot would most likely only be partially functional due to the stiffness she already has. She would still have a smaller foot requiring two different size shoes the rest of her life.
  • Because of the intensity of all of these surgeries, we will have to focus a lot of our time and energy into helping Siena get through this and there is the potential for the other kids to feel left out or ignored. They might feel resentful towards Siena

  • Siena would likely miss months of school while undergoing the lengthening surgeries.
  • Siena would likely wear a platform shoe on her right side due to the leg length discrepancy which may lead to teasing and bullying.



After months of research, prayer, meeting with doctors, etc. we feel like we finally got our answer after we met with our last doctor at Shriner's on Tuesday. After looking at the x-rays and analyzing Siena's feet and legs, she was able to explain with great detail and clarity the extent of the issues that Siena has. She explained the intense path we would have to take if we went down the route of limb lengthening. Siena's case was much worse than we had previously been told. She said that if it were her child, she would amputate as well (she told us she was holding back her bias and desire to tell us this until we had shown that we were heavily leaning towards that direction). In that moment, it just felt right and we felt like we had all of our questions and concerns resolved. We felt peace about the decision and set up an appointment to have the amputation surgery on June 18th. The timeline for all of this is pretty simple. We would go in for the surgery on June 18th. Siena would stay in the hospital for 2 days/nights while an epidural keeps the pain under control. Then she would be put in a cast for 5-6 weeks. After that she would be fitted for her first prosthesis. A couple weeks later she would get her prosthesis and do a few physical therapy visits to get used to walking in it. She should hopefully be ready to go for pre-school in the fall if all goes as planned. Down the line we will likely need to do a minor knee surgery to correct the knock knee and do a growth clamp on her femur on the longer leg to correct some of the length discrepancy. All of the other issues shouldn't be an issue since the amputation would remove the foot and correct most of the limb length discrepancy.

Siena after her x-rays.. she was picking out a sticker

Our awesome Dr!

Just chillin...

We got a tour of the prosthetics area. Super cool!

That is a summary of a lot of what we have learned and thought about over the last several months. Lots of people have given us their opinions and we have struggled to decide what we should do. We have gone back and forth many times but we feel good that we have decided that for our family and for Siena, the best option would be to do the amputation. It's an excruciating decision we have had to make. Siena is aware and although she is a little scared you can tell she is going to handle it so well. She is just excited to put some cute fabric on the prosthesis (she has already picked her next 6 years of prosthesis designs). She is also convinced that the prosthesis will make her run really fast and jump really high and make her "tall to the ceiling". She also wants the prosthesis to have a really big foot so she can wear mommy's shoes. We have kept her involved in the decision making process and believe that she understands to the extent that a 3 year old can that this is the right path. This has been one of the toughest decisions we have and probably will ever have to make. We have the blessing of knowing God's eternal plan and know that Siena has great things in store for her and one day will get her foot back. We have each written Siena a letter for her to read later on in life so she can understand why we chose this route for her and how we came to our conclusion. Hopefully this helps if/when she is struggling as a result of the amputation.

I also want to add that Joel and I are very grateful that Siena is a healthy little girl and that it was only her leg that has issues. Anytime we start to get too in our heads with it all we remind ourselves that things could be much worse and we are grateful for her overall health.

Let us know if anyone has any questions about Siena's case, the amputation, how we made our decision or anything else. We are happy to talk about this and share anything that might be helpful to others.

Thanks for the support!

Joel and Kristin

Monday, April 6, 2015

Home Remodel – Kitchen and Living Room Redo

If I was to say that I have had fun remodeling my house, that would be a MASSIVE understatement! It took a fair amount of time to get it to where it is now, but I LOVE it! There were so many late nights, set backs and budget surprises, but it was well worth it all. It all came together exactly like I imagined it in my head.
When we were searching for a house to buy when we moved out here to Utah I had a pretty specific location we wanted, so it all came down to finding a good house. After searching around on for a number of weeks we settled on 3 different houses that had pretty much the same layout. These houses were ideal for the open layout I had envisioned for our next house. When our realtor took us to the houses however, the last house (the one we bought) was the clear winner. When we went through the other two houses I couldn’t help but notice that EVERY SINGLE ROOM needed some updating and got pretty overwhelmed. When we went through this house, all I could think was WHEW! This one is at least safe and livable the way it is.

Shortly after moving in (I believe it was less than a week later) I started calling our contractor and had him come out to look at taking down the wall between the kitchen and the living room. I also had him quote us on moving cabinets, ripping out tile and installing new baseboards, etc. He had awesome prices so I was able to do more than I thought we could.

Joel and I had the privilege of tearing down the drywall on the wall between the kitchen and livingroom -> which was a blast! we also installed our back-splash, installed the floating shelves, the new glass door over the microwave, did LOTS of painting, installed the mantle on the fireplace, and many more projects. It kept us busy to say the least, but I’ll say it again, it was worth it!! Here are some of the before and afters





I love my blue door!:)





Before (when we were moving in):


And just a few more for fun:

Anyway! All the stars finally aligned and we were able to keep the kitchen/living room clean long enough to get a few shots. It’s been fun thinking up ideas and watching them come to pass. Hope this can inspire your own home-reno thoughts!

Monday, March 16, 2015

Update on Siena's foot at Shriner's Hospital

The last time we had an appointment with Siena's foot doctor at Primary Children's, we were told that amputation was still on the table and may even be a good option. Since then (about 2 weeks ago), we've been doing tons of research and pondering on what to do. We found some great resources on line and talked to a family here in Utah who has a double amputee child in the family. We had started feeling that an amputation might be the best course of action. We heard about Shriner's hospital which is a non-profit hospital that runs on donations and we found out that 100% of our health care costs related to Siena's foot would be covered by them. And it doesn't hurt that they are experts at the conditions that Siena has. We've been anxious / excited for today so we could meet another doctor and explain our situation and thoughts about the whole thing. We were also excited to find out another perspective about Siena's foot/leg. Below are some new things we found out at this appointment:
  • She currently has about a 3cm difference in her leg (just over 1 inch). Based on some calculations using the x-rays we got from Primary Children's, that difference will grow to approx. 6-7 cm (about 2.5 inches). This is pretty mild relatively speaking and would most likely only require one leg lengthening if we did it when she stops growing (around 12-14 years old).
  • Her femur is about 1/2 inch shorter so they would most likely do a minor surgery to attach a "clamp" on the growth plates to slow the growth on her "good" foot at some point by 1/2 inch to correct that problem and ensure that her knees were at the same level.
  • Her Tibia and Fibula are short as well but both bones are at least present which is a plus. Due to the shortness in her bones, she has fairly bad knock knee which is where the knee bends inwards and that will need to be corrected as it gets worse. It's a fairly simple surgery where a plate/screws straightens her knee out as it grows.
  • She does not have an ACL. Apparently that is very common for fibular hemimelia patients. It shouldn't hinder her functionality though. The body should learn to work without it from what we understand.
  • Due to her foot/ankle structure,  she will have a ball and socket ankle which could make her more prone to ankle sprains later in life.
  • Now that her casts are off and her Achilles tendon is stretched back out, they prescribed a Ponseti brace (see pic below) to wear every night for the foreseeable future. This will keep her foot stretched in the right direction and help her foot and ankle grow correctly. I'm fairly excited that they gave us these types of shoes because the dobbs brace that we had when she was little was nice for movement's sake, but these look like they will be more comfortable and she will have WAY less blisters with these.

  • They gave us some orthotics to use in her shoes which will help correct some of the length discrepancies and allow us to use the same size shoe since her orthotic will lift her foot in the shoe and make it fit more snugly.
  • The Dr. feels like Siena is a great candidate for a limb lengthening due to the mild nature of her case. She has a good foot and ankle (as far as she can tell right now) and the length difference isn't too drastic. She doesn't think it would require too many surgeries over her lifetime to correct this. And she believes her foot and leg should have good functionality when all is said and done.
At this point, we need to keep doing some more research to make sure we don't want to go the route of amputation. If you do a fair amount of research like we have you will find that amputation vs going the limb lengthening route is a pretty big gray area. It's hard to say which will have a better quality of life. We will continue to have appointments every 6 months to re-evaluate and do x-rays, and get new braces for her ponseti brace, etc. We are glad to have some additional information but we still feel fairly conflicted on what's the best path forward. That's about everything we can remember from the meeting with the Doctor. Until next time...:) Below are some pics too!

Siena playing with the chipmunks before her appt:

 Siena getting the cast removed. She did great!

Siena walking around in one of the Ponseti braces. She thinks they are cool shoes...

Sunday, March 15, 2015

Middle-end of February 2015. Club Foot/Fibular Hemimelia Appointment

I love sleeping baby pictures. Here is a cute one of Declan all burrito-ed up!
I curled Siena's hair with a curling iron for the first time a few weeks ago for church. She loved getting her hair done! Turned out pretty darn cute too if I do say so myself.
Joel got a new phone and it had capability of doing slow-mo videos. When testing it out we got an epic video of Siena. She kept saying that it was burping and mooing when we watched it back too. So funny

We went to a trampoline play place with the kids a few weeks ago. The kids loved it! Even Brighton held his own against the big kids.
Can you see this next one?!:) I laid Declan down on Siena's bed and asked Siena just to keep an eye on him while I laid Brighton down for his nap in the room next door. I came back to this sweet sight. Siena had tucked him in the blankets, given him a stuffed animal to hold and was reading him a book. Cutest big sister award!:)
Such a sweet little guy!

 This video is just a slow-build of Declan about to cry. I thought it was too cute though and had to post it.

 Everyone loves Declan!:)
 I love when they play well together. It's so sweet!
Siena loves listening to her books on CD that we get from the library. I also love it because I am able to get a few things done.
Declan cooing.
 Knock knock..
 Bippidi Boppidi Boo:)
 Siena had a club foot/ fibular Hemimelia appointment. She has these about every 6 months, so I was looking forward to seeing if they had anything new to say. Brighton was a fan of the toys they had in the waiting room.
I took this picture and sent it to Joel telling him about how they had put us in a room set up for doing the castings for club foot that we had done with Siena when she was a baby and how it was bringing back such "fun" and then about 2 minutes into the appointment the dr said Siena needed a cast. Talk about some negative foreshadowing!lol Siena's right leg is shorter than her right and she had apparently been walking on her tippy toes on her right leg and that had tightened her Achilles on that leg and it needed some stretching out. They stretched her leg and then put a cast on it for a week to stretch it out. They wanted Siena to come back for new casts once a week until it was better so we made 2 more appointments for the next two weeks just in case. More on that later in the post.
 She did great getting her cast on. The Dr was really impressed and said she does better than even the
 She's was quite a fan of her pink cast
Brighton LOVES "Deck-uh" (Declan). If Declan is awake and I'm not feeding him Brighton is usually right near him. It's adorable!
 Snapped this sweet picture of Brighton's chubby cheeks! Love them!!
 He just recently found his belly button and thinks it's the coolest thing ever!
 Brighton says quite a few words now. One is "here you go" which sounds more like Hee-a-go. It's so cute.

 About 2 days after getting her cast on she was complaining that it was hurting a ton and wouldn't walk on it.
 Sweet tuckered-out baby!
 After a day or 2 of her complaining and occasionally bursting into tears we went back in to the office to have them take off the cast and check to make sure there were no blisters or problems with it. After determinng that it was fine and that it was probably just her Achilles stretching that was hurting they put on another cast and we made another appointment for a week from that day to get that one off and the next one on.Siena was pretty excited that she got to get a new color. She chose red for her 2nd cast. More to come on that in the next post.
I posted a shorter version of this video on Instagram and said this: "Oh my goodness I am dying over this video! I think I have a little OCD boy on my wish I could have posted the whole video.. He was pretty upset over that darn trash can lid that would not stay closed!"


This is how we make it through church my friends.. two fistfuls of
Siena has been learning songs in Primary and it is so fun to hear her singing these songs at home. The most recent one is called Gethsemane.


Brighton was so upset that morning that he couldn't figure out how to use his spoon. Overall he did well with it though.
 Siena loves having Brighton walk to her. I'm still working on teaching her that you have to stand a few steps away for him to actually be able to walk to her.:)

 Hanging out with his bro.
I love Brighton's car sounds:)

Brighton's first time-out. It was too cute to not take a picture! He sat there really well too. I guess he's seen his sister do it a time or two or
Shortly after Brighton went to time-out Siena had a meltdown and had to go to her room until she calmed down. I took a video in which you can hear Siena screaming from her room and can watch Brighton's reaction to her freakout. It was pretty epic..
I was also lucky enough to have my friend, Amanda, from college come to visit with her sweet little girl. Her daughter was such a fan of Declan, it was hilarious!