Hypochondroplasia - limb lengthening - surgery

The plan for Fi was to have surgery and lengthening on all 4 segments of her lower limbs at the same time, this halves the time spent in wheelchair, hospitalized, out of school, doing daily physio, etc. along with improving the overall proportionality.

Based on the size of her femurs and the lack of angular deformity, we knew she could get the internal fixator, the Precice Nail in her femurs. This means no bulky external fixator, no pin site infections, minimal scarring.

These can't be inserted into tibias of children still growing though, so she had to get externals on her lower legs, Taylor Spatial Frames (TSFs) which also helped with some minimal angular corrections that she needed.

A couple months before the surgery we traveled to meet with the surgeon and his team who discussed the plan with us in detail, we got to ask all our questions. Fi had a number of them as well and they all got addressed.

I think the thought of your child in pain is the most daunting for people. It certainly was for me. I will write about this in detail, but I can say that other than a few bad minutes during therapy she has only taken Tylenol following the surgery, once a day. Slept through every night and has been in a great mood - so all our expectations have been surpassed.

The surgery took nearly 10 hours and Fi's surgeon was able to do everything he planned to do. She had a wonderful anesthesiologist who listened to her tonsillectomy GA story and decided to change his approach and went with the TEVA approach for GA. They also did 4 peripheral nerve blocks at the very end of the surgery to manage pain. She woke up smiling in no pain.

She spent 3 nights in the hospital's pediatric ward, where they have a fantastic group of nurses, who know all about fixators and post op care for ortho patients. Every 12 hours we would get a new nurse and I always felt that nurse was the best nurse I have ever met in my whole life. Wonderful experience throughout.

Once the nerve blocks wore off, she was briefly put on a PCA on day 2, but it was making her super drowsy, so that was promptly stopped, she was given 2 stronger painpills (opioides) but we realised we didn't really need them, so we stopped and we went to our accommodation, the local Ronald McDonald House (more on them later) on Tylenol.

During the hospital stay the biggest things were for us to get used to the fixator, learn about pincare, learn the PT exercises we had to do several times a day from day 1 on and learn to transfer to the wheelchair from the bed and back and to the commode. The inpatient PT and OT came to see us every day and they were full of great advice.

Next chapter.: our experience and helpful hints fr the distraction/lengthening phase.