Daniel's Steps - Proximal Femoral Focal Deficiency

Today we got the news that after 11 months, Daniel can have his fixator removed on September 10th. It has been so so long since my last update so I thought I should update Daniel's blog. I have been using Facebook to update more recently so not using the blog as much. But, I want to make sure I update it for Daniel to read when he is older and also help other families going through this process in the future.

So, when I last posted at the end of February, we had finished lengthening. We had to wait a few weeks so that Dr Paley's team could make the adjustments to the external fixator to change the angle of his bone. One day during Spring Break, we met Tony (the fixator fixer) to make the adjustments. It was a very long day for Daniel, he had to lay on the table for 8 hours, a long, long time for a 6 year old to sit still. It was pretty traumatic for him when they had to cut the big bar that runs along his femur with a wire saw but he was so brave and such a trooper during it all. The iPads ran flat and he tried everything in the candy machines but we got there.

Eight hour epic fixator adjustment

Unfortunately, the next morning, things didn't look or feel right. We took him straight up to Dr Paley's office for x-rays. The fixator had collapsed due to a miscommunication as to which type of hinges should have been used. The fixator was adjusted again but unfortunately we had lost about 2cm of length which was devastating because it had taken us so long and so much pain, sweat and tears to get that 2cm.

We had to turn one of the sections of the fixator for about 10 days to change the angle of Daniel's bone. Once this all lined up properly, Dr Paley suggested some further adjustments so we could lengthen again to regain the length we lost. Fortunately, we were able to turn 4 times a day (1mm a day) so it wouldn't take as long. The best news was that we didn't need to use the knee bar at night which made a HUGE difference to Daniel's pain. He wouldn't have been able to stay at school if we had to use it.

The good news is that we were able to continue lengthening beyond what we lost so we think we ended up lengthening about 10cm! Both Shane and I are still a bit confused as to what length we actually achieved in regard to the shoe lift. With the changes in angles and stopping and starting, even the PAs had difficulty working that one out. But at the end of lengthening (for the second time!) in mid-May, his shoe lift was measured at 8cm.

Done Lengthening!!

So since mid-May, we have been waiting for Daniel's bone to consolidate and today we got the news that we can book in removal for September 10. We are all very excited to say goodbye to the fixator. Daniel will have a non-weight bearing stage of about 4 weeks before he can resume normal activities. At the moment we are going to PT twice a week. Daniel is getting 65 degrees on his knee bend and we are not really getting any more than that despite bending at home and PT. His therapist is hoping that once the fixator is off, we will have some more gains. The fixator is like a cage around his leg now which makes it difficult.

Daniel finished First Grade in June, enjoying the last week of festivities. He has had an awesome summer and has not used his walker or wheelchair as his pain has improved. We use the scooter now to get around. Next lengthening we will definitely use a scooter again, works so much better than the wheelchair or walker.

My parents and best friend visited from Australia. We went to New York City and caught up with some Aussie friends there. He has been to drama camp, art camp, science camp and Lego camp. We have been to the baseball which he is really into now since playing it with his therapist, Don, every PT session. With the Paley kids, we visited the zoo, the Science Center, NOVA nights and a photo shoot for the Paley Foundation with all the kids on bikes.

He is starting back at school next week with Max who is starting Kindergarten.

Max graduated from Pre-K in June, we are so proud of him. As well as starting Kindergarten next week, Max is starting soccer and swimming lessons soon and we are looking forward to Daniel joining in very soon! Max has been an awesome little bro to Daniel, he best friend through all this.

We have all really loved living in Palm Beach and will be so sad to leave. The generosity of people has been incredible. We have also got to meet so many Paley patients and feel very much part of a community of incredible families. I think Daniel will look back at his time here fondly which will help when we return to Florida in 4 years time.