BEST FOOT FORWARD
Josh Adam was born with only three toes on his right foot and no ankle. His parents faced a tough choice. Today he is an active youngster and a keen sportsman.
When you first encounter Josh, all you’ll notice is a friendly, outgoing nine-year-old boy with an impish smile. He’s fiercely protective of his younger brother Dian, and he recently won a prize for coming second in his class at Gene Louw Primary School in Durbanville, Cape Town. As he chases around the garden after a long day in the classroom, you would never suspect he has overcome several difficulties in order to stay healthy and active.
When expectant parents Chester and Juanita Adam went for a routine 4D scan at 20 weeks, they were dismayed at the results. “Doctors picked up something wasn’t right with our unborn son’s right fibula,” dad Chester recalls. The next day, a foetal care expert confirmed the news. Josh had fibular hemimelia, a rare condition with no known cause. “It was a massive blow to us,” Chester says. “I’d always imagined my son as a little sportsman because action is a big part of my life – from club cricket to road running. Now we were faced with the idea of our child not even being able to play with friends, let alone sport; it was devastating.”
Fibular hemimelia
Mediclinic Louis Leipoldt orthopaedic surgeon Dr Ryno du Plessis, who has treated Josh since birth, explains that fibular hemimelia is a congenital condition of the leg that affects about one in 100 000 people. “The deformity is complex, and it affects the limb in five ways,” Dr Du Plessis says. “These include leg length discrepancy; foot and ankle instability; bowing of the tibia (shinbone); genu valgum (knock knees); and knee instability. There are four levels of severity with varying combinations of the above problems.”
Still reeling from the shock of the diagnosis, Chester and Juanita were referred to Dr Du Plessis, who carefully explained their options. “We went to see him while Juanita was still pregnant because we wanted to be well prepared for what lay ahead,” Chester explains. “Dr Du Plessis was so positive about Josh’s future that we both felt so much better about the hand we had been dealt. Now we just needed to decide how to play the cards.”
Tough decision
The concerned parents had a difficult decision to make. “We basically had two options,” Chester says. “We could either opt for amputation [a portion of the lower part] of Josh’s leg and a prosthetic – or leg lengthening, which would entail wearing a frame and brace for several years.” Dr Du Plessis explains further: “We needed to make an early decision between a reconstruction pathway or an ablation (amputation) of the affected limb. Both have pros and cons, and a quick fix simply does not exist.”
Limb reconstruction entails multiple operations. “It’s typically done with the use of [computerised] hexapod or Ilizarov external fixators; sometimes combined with the strategic halting of the growth of the non-affected limb at a later age,” Dr Du Plessis says. This obviously affects a child’s social and emotional development as they’re unable to participate in many activities. “While the child is maturing, four major interventions are sometimes necessary and varying functional outcomes are to be expected. Sometimes it’s also necessary to do an arthrodesis (fusion) of the ankle joint to create stability.”
On the other hand, the benefit of an early ablation (amputation) is that the child grows up without a psychological scar if done early. “The specific ablation that can be offered makes use of the heel pad so that the stump is end-bearing, meaning prosthesis fitting is well tolerated,” Dr Du Plessis explains. “The other obvious pro is that the multiple interventions described above aren’t necessary.”
“He simply performed like any other toddler – wobbling a bit as he learned to walk. When he wore long pants, it wasn’t even noticeable that he had a prosthetic limb.”
Chester Adam
Heartwarming outcome
After much soul searching, the Adam family opted for early ablation. “Josh was born with only three toes on his right foot and no ankle,” Chester says. When he was 13 months old, he underwent the amputation. After two months in bandages, he started walking around on his new prosthesis. “It was so heart-warming,” his father says. “He simply performed like any other toddler – wobbling a bit as he learned to walk. When he wore long pants, it wasn’t even noticeable that he had a prosthetic limb.”
Today Josh is a keen sportsman. He swims, skateboards, plays cricket and rugby and participates in athletics. He has never felt different from his peers and happily refers to his prosthetic as “stompie”. One challenge is the fact that he’s still growing, says Juanita. “Depending on his growth, he gets a new prosthesis about once a year. But the feet are another matter. He needs a new foot about every three months because he literally runs through them. The prosthetist has started adding a durable layer to the bottom of the foot to strengthen it a little.”
As Dr Du Plessis explains, his patient is currently on crutches because of two recent operations – an osteotomy of the tibia to correct the anterior bowing, and a rotational correction of the femur. An osteotomy is a surgery where diseased, damaged, or injured bone is cut and reshaped to improve function and reduce pain. “Both the osteotomies are now healing at the same time,” Dr Du Plessis adds. “These operations will correct the biomechanics of walking and running and make prosthesis fitting easier. And help make Josh an even better little sportsman!”
