William was born with a rare disorder called ‘Congenital Pseudarthrosis of the Tibia’.
This became apparent when William was 7 months old. He had not long learned to roll. He rolled from his tummy to his back when his Tibia spontaneously fractured.
I called an ambulance as his cry (as you can imagine) had me distressed and frightened.
X-rays were taken which revealed a fracture in both his Tibia and Fibular (right leg). He was cast and sent home.
At the 2 week check up X-rays showed that the bone was not healing and was abnormal. We were then referred to an Orthopeaedic Specialist at the Lady Cilento Children’s hospital. This is when we found out William had CPT (Congenital Pseudarthrosis of the Tibia).
He was cast for an AFO (Ankle Foot Orthoses) to keep his leg stable.
When William was 17 months old (November 2015), he had an operation to stabilize the Tibia. This consisted of removing the diseased bone and inserting a rush nail from his knee to ankle. He still had to wear an AFO to protect the Tibia from refracturing. There was only a 20% chance that his bone would unite and heal.
Over the next 6 months William had 3 monthly reviews. The X-rays showed that there was some unity happening.
William wouldn’t put any weight on that leg nor would he like to straighten his knee.
We continued with Physio.
I took him back for a check up. X-rays showed he had ‘wipering’ of his lower Tibia (the rod was moving and widening the cavity of the bone making it thin and brittle). They also suspected an infection.
At 27 months old (August 2016) the surgeons decided to remove the nail. Tests were done and an infection was present. They then left the rod out and a strong antibiotic was given to kill the infection.
William has been in a KAFO (Knee Ankle Foot Orthoses) for the last 9 months. He has had fortnightly physio and still not walking unaided.
March 2017 we had another routine check up. They discussed more operations of which had not yet been successful and then there was Amputation.
CPT is a rare condition. It occurs 1:250,000 births. Australia has a population of almost 24 million. 6 million; 25% are children. Therefore there are only 24 children of all ages in Australia with this condition. There are more than 24 pediatric orthopedic surgeons in Australia. With only this small number of children spread out across Australia how can any one Pediatric Orthopedic ever get enough experience to treat this condition with a high degree of success.
In my search for a more definitive solution, I came across a group of Australians who have travelled to Florida to see Dr. Paley.
I was put in contact with the Paley Institute where Dr. Paley responded personally and reviewed Williams X-rays. He confirmed the CPT and put forward his treatment plan.
Dr Paley has been treating patients with CPT for 28 years. He has treated over 150 children SUCCESSFULLY with this rare condition since 2007. His success rate has been 100% with 0% re fractures.
He had gotten every patient to heal in 3 – 4 months.
This is a very technically demanding procedure which requires great attention to detail and much experience. You only get one chance to get it right, solve and eradicate this disease.
I was told William is at an ideal age for surgery. The sooner this is done the less risk there is of secondary changes. Due to the effect of the growth plate, Williams leg is approximately 6 cm shorter. His femur on the CPT leg is compensating for the discrepancy and growing at a faster rate compared to his other leg. His foot is also effected and more than 3 sizes smaller. Leaving this operation any longer will result in more invasive lengthening procedures.
The success rate of treatment of CPT in most people increases with age but is nearly 100% failure at age 3.
The only exception to that is Dr. Paley’s experience and results. No other surgery has shown consistent union achieved in patients under the age of 6. Most of his patients were treated between the ages of 1 and 4 years old. All united and remain healed.
The only downside to treatment with Dr. Paley is the distance and cost involved. This is overcome by the upside of his success rate, peace of mind, and safety.
We cannot achieve this on our own.
To have this surgery in Australia based on the current experience with this disease is likely to result in multiple, invasive and painful surgeries throughout his formative years which in all probability could end in a amputation.
William has just started walking in the last 4 weeks (October 2017). Due to the pressure and movement his leg is deteriorating rapidly……….