Maya

Content Area

Maya was born in January 2002. She appeared to be a perfectly healthy baby. The middle toe of her left foot, however, struck my attention right from the start. It was thinner and smaller than the other toes. When I pointed this out in hospital I was told that this resulted from being cramped in the womb. Later on, I would get to know that this was a sign, one of the smallest indications of a rare condition known as fibular hemimelia.

When she was 7 months old, I noticed that her left foot seemed thinner than the right and her leg appeared shorter. I immediately had her seen by a local doctor who dismissed my concerns as obsessions and stress. I insisted on taking x-rays against doctor’s orders. They confirmed my ‘’obsessions’’ – that Maya’ s left leg was shorter than her right. I was referred to neurologists and orthopedic surgeons who could only tell me to wait until she was older because they could not diagnose her condition. I was told that the leg length discrepancy could get worse, or perhaps her left leg would eventually catch up. So we were meant to wait and see and hope for the best.

I researched leg length discrepancy and all the information indicated rare conditions that no doctor in Malta was willing to identify.

At that point I decided to take Maya to London for a consultation with a top orthopaedic surgeon. He diagnosed Maya with congenital fibular hemimelia. The discrepancy would reach 12 cm upon maturity. They could fix it the best they could however she would always walk with a limp and the end result could not be guaranteed. They would start the procedures when Maya would be 8 years old. Until then she would have to wear shoe lifts. This meant that for most of her childhood, Maya would not be able to live a normal life. She would not be able to participate in most physical activities. She would not be able to walk properly without wearing a lift in her left shoe. Most of all she would feel different.

This was unacceptable. After more extensive research I came across Dr. Paley. I printed and studied a good part of his documentation. At the end of it I was convinced that I had to take Maya to see him.
I emailed him and was pleasantly surprised to receive an answer from him immediately.

A visit was scheduled immediately and we made our way across the Atlantic to Baltimore. Dr. Paley was direct and very sure of his diagnosis. Maya had Fibular Hemimelia and Congenital Femoral Deficiency. Her fibula was shorter, her knee was in valgus (this meant that from the knee her leg tilted outwards.) The structure of her ankle was completely different to what it should be like. According to Dr. Paley, it was one of the most abnormally formed ankles he had ever come across at the time. Additionally her left femur was shorter than her right. This in turn affected the left side of the hip which was not as ‘locked in’ as the right side. We were immediately given a procedure which Maya would follow throughout her childhood in order to achieve a good result by full bone maturity. What impressed me most was the fact that Dr. Paley was so sure of both the diagnosis as well as the solution options. Up until then, we had not been given any straightforward answers. Now our decisions were based on something definite. The best thing of all was that in the USA the treatment was much more aggressive than in Europe. They also believed that the first treatment should take place before the child is 4 years of age. Maya would need two major surgeries, one at the age of 3 and one towards the end of bone maturity which meant around the age of 12/13. And a few relatively small ones in between. This was great news. It meant that for the largest part of her childhood, Maya would lead a normal life.

The first years were tough. Maya had to wear a shoe lift from the minute she started walking (eleven months old) up until the first surgery. She also had to wear specific ankle booties, to keep the ankle securely in place as much as possible due to its abnormal structure. This was a challenge, particularly in the hot summer months.

At the age of 3, Maya bravely underwent her first leg lengthening process and ankle reconstruction with the Taylor Spatial frame method. The lengthening result was perfect. We stayed in Baltimore for three months and followed up with several visits over the following months. Maya gained 5 cm in length. Dr. Paley over lengthened the fibula so that she would not need to lengthen the fibula again in the future and the femur could eventually be addressed. Whilst the majority of patients had one large discrepancy in one part of the leg, fibula or femur, Maya had a smaller discrepancy in both. An 8 plate was inserted in the knee to correct the valgus. This would be removed a couple of years later. The ankle reconstruction procedure proved to be very difficult. Even though Dr Paley locked the ankle, it ‘slipped back out of place’ shortly after the fixator and cast were removed. (The following year, Dr Paley went through another attempt at reconstructing the ankle, again by using an external fixator, however the ankle showed a lot of resistance and the same problems persisted. (This would be taken care of again at a later stage with a new technique).

Dr. Paley followed Maya’s progress, following our return to Malta. He even kept me informed of his trips to Europe so that in between some seminar here and there, he would see Maya and keep track of how her leg was growing. We would send him x rays from time to time to see how her leg development was progressing.

As a result of her first lengthening Maya could do away with the shoe lifts and join her friends in ballet and football. It was amazing to see how the other muscles and ligaments of the foot and leg compensated. I will never forget our first trip to buy normal shoes in Baltimore. We bought quite a few pairs !

Maya has many happy memories of all our trips to Baltimore and does not recall anything negative connected to her surgeries. One of the main reasons why Dr. Paley operates at a young age is for the young patient to forget the physical pain and to limit the psychological trauma.

Maya’s next surgery to address the femur and the ankle was scheduled to take place four or five years later. We met up with Dr Paley in Sicily the year before her ankle surgery for a final consultation. A year later we flew to West Palm Beach Florida where Dr Paley had relocated. This time he inserted a pin in the ankle on one side to correct the ankle as the bone grew. This would basically stunt the growth from one side, in turn turning the ankle to the right position. We flew back home and starting witnessing the miracle that was taking place. There were a couple of years to go for the ankle to move to its normal position.

Our final plan was to have the last biggest surgery, lengthening of the femur. By now Dr Paley had created a new revolutionary technique, the PRECICE method and Maya was a perfect candidate. Having financed all of Maya’s surgeries alone (congenital conditions are not covered by medical insurance in our country), Dr Paley very kindly offered to fly to Malta. This way we could save up on hospital surgical fees. In June 2014, the Precice nail was inserted in Maya’s femur bone. He also inserted another 8 plate in her knee as her valgus was back. A week later we flew to West Palm Beach to start lengthening and therapy. We would lengthen .25 of a millimeter three times a day. Therapy was daily at the Paley Institute.

The procedure worked perfectly with no complications. I must admit I was very nervous as they calculated the estimated leg discrepancy at the end of bone maturity as this would be Maya’s last surgery so we had to get the length right! It is amazing to see how Dr Paley and his team could determine all these measurements so accurately considering the fact that Maya still had two years of growth.

In July 2015 we took our last emotional journey to Florida for Maya’s final surgery. This consisted of the removal of the knee 8-plate and of the amazing device in her femur which had lengthened it to perfection. Maya had grown considerably taller during this past year and Dr Paley’s calculations had proved to be correct. She now stood tall and straight with both feet firmly touching the ground.

We are eternally grateful to Dr Paley and his team who are so dedicated and committed to giving the best result to all their patients from all over the world. They have revolutionized limb deformity techniques and they have adopted the most aggressive methods to treating all kinds of rare conditions and they are so successful.

When Maya was 8 years old, she wrote an essay at school about her hero. Without hesitation she chose to write about Dr Paley.

Maya is 13 years old. She swims competitively and she walks tall and strong with no limp.

Dr Paley gave my daughter an amazing childhood and the possibility to have a normal life. He is a creator of miracles and he is our hero.

Claudine
Rabat, Malta