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|
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.
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