We were becoming more comfortable with Emma’s diagnosis of Fibular Hemimelia and we did more research on the Internet and networked with other physicians. We made a decision to have Emma seen by several orthopedic surgeons so that we could have multiple opinions to help us determine what was going to be the best for our daughter and our family. This is a very personal decision that must be based on many different factors, including access to medical treatment and especially the severity of FH.
Now it was time for a second opinion. We met with Dr. Woo, a highly distinguished pediatric orthopedic at Florida Hospital in Orlando. After evaluating our daughter, he too recommended amputation. Dr. Woo did acknowledge that we should do further research and did give us names of other orthopedic doctors who were doing other procedures including ankle reconstruction and limb lengthening. However, he was unsure how they got such favorable results because he felt their studies showing success could not be reproduced by other facilities. Dr. Woo felt the problem with limb preservation through ankle reconstruction (Super Ankle Procedure) was not a good option because of the poor outcomes he had seen.
Dr. Woo’s other option was not to do the amputation but instead to fuse the ankle and then continue with the limb lengthening. Fusion sounded great at first, because there would be reduced chronic ankle pain that Dr .Woo mentioned you experience from the ankle reconstruction. You also address the limb length discrepancy that FH patients have because you continue with the lengthening procedures. However, as my husband and I thought about it, it didn’t really make sense to us. If you fuse the ankle, it is locked in a neutral position. This allows for ankle stability, which is a big part of the ankle problem in FH; but if it is fused, you can’t point your toes down. How would she ever be able to get on a pair of pants? It makes for a very poorly functional foot. This seemed like an even less favorable treatment than amputation in our mind, so we decided this option was not right for our daughter.
In summary, Dr. Woo’s advice was to amputate.
As these opinions were coming in my husband and I were trying to become more comfortable with amputation; do research on various prosthetic specialists; as well as the surgery or surgeries needed to do the amputation.Next appointment was with the Pediatric Orthopedics at Arnold Palmer Hospital, Drs. Albright and Price. They too recommended amputation.
The next appointment was with Dr. Dror Paley, who was previously associated with Dr. Herzenberg and Dr. Standard at the Rubin Institute of Advanced Orthopedics at Sinai Hospital in Baltimore. He had relocated to West Palm Beach, FL so living in Orlando, this was right in our backyard.
My family and I drove to West Palm and had an appointment with Dr. Paley. We went with skeptical thoughts because of all the Orlando doctors’ cynicism about what they felt was an overrated “superankle” procedure they could not master.
With that in mind, we met with Dr. Paley and left his office with guardedly excited optimism. He was very knowledgeable and spent a long time discussing FH, the ankle procedure, and future surgeries, unlike what the opponents of limb lengthening say, it doesn’t mean your child will spend his or her whole childhood in a hospital.
When asked what he felt Emma’s severity was on a scale of 1-10, he said a “3”. This gave us hope!
Throughout all of these appointments, Emma had her regular well child appointments with her pediatrician, Dr. Middleton, and it was he who gave us the best piece of advice. He did not give us advise on how to handle our daughter’s medical care, he gave us support. He said, “Right now you are in a fact-finding search. Take it all in and one day, there will be a moment where it will become clear and you will know what is the best option for your daughter and your family. There will be a level of comfort that you will have and you will know it is the right decision, whatever it may be”. This was the best thing anyone told us because it was true and did happen for both my husband and me.
For us, this comfort level was found with Dr. Shawn Standard and Dr. John Herzenberg at Sinai Hospital in Baltimore.
We originally had an appointment with Dr. Herzenberg however Dr. Standard stopped in to say hi and he ended up staying and talking with us for 2 hours. It was amazing. He explained FH again, because no matter how many times I heard it, it didn’t hurt to hear it again. Dr. Standard discussed the future procedures we could anticipate should we go ahead with their recommendation of ankle reconstruction and limb lengthening. Dr Standard showed us pictures of children with external fixators and before and after X-rays. Marilyn, the child life specialist, even introduced us to other families who were there. Dr. Standard said that children typically have the first surgery around 12-18 months, which consists of ankle reconstruction (super ankle) and tibial straightening. Then the first true lengthening at 3-4 years old and possibly another around 10-14 years old but the exact timing of everything would depend on her growth and limb length discrepancy.
Based on both information sources, Dr. Paley and Dr. Standard, Emma would have approximately 7-12 cm of limb length discrepancy at skeletal maturity based on her height projections at our initial visits. Just as Dr. Paley said this was a relatively mild case, both Dr. Standard and Dr Herzenberg also said she was a 3 out of 10 on the severity score.
We finally had our moment of clarity as Dr. Middleton said we would and it was during our appointment with Dr. Standard. He and his staff made us feel so welcomed and a part of their big family. We left that day without any doubts in our minds of what was best for our Emma.
Funny thing happened as we were leaving the appointment. There was relief. It was like no one, neither me, nor my husband, or either of my parents needed to discuss what to do next…we all just “knew”.
We cancelled our remaining orthopedic appointments!