Sep 11, 2017 Latest post:
Jul 28, 2018
Those who know and love Chris have been with all of us through this journey - since we were first trying to have him diagnosed when he was two, learning there was no treatment at that time, the surgeries, the risks, the clinical trial with Bio Marin, the weekly infusions and the ongoing struggles but most of all the ongoing story of bravery and a personality that will make you smile EVERY day.
Chris has Morquio Syndrome. Otherwise known as MPS IVA. We encourage you to check out www.morquiosity.com to learn more about it.
Chris had been having more and more issues with his knees/legs and began to use crutches full time almost two years ago. We began to seriously explore surgical options for his legs last year. We've been in conversations with his Mayo team for years about the possible options, but we also sought out a 2nd opinion from Dr. Mackenzie at DuPont Hospital for Children in Wilmington Delaware in November 2016. Dr. Mackenzie and his team at DuPont agreed with the type of leg surgery being proposed by the Mayo team. However, they had recently become aware of an issue with many Morquio individuals involving the trachea and completed a test on Chris to review his trachea. They found that his trachea was indeed "kinked" and/or twisted for lack of a better description. This can be a life-threatening situation and a bit of a "shock" for us as we had no idea! Chris did not (still does not) display the normal symptoms of this condition - such as sleep apnea, snoring etc. We brought all of this information back with us to Mayo and in April met with Chris' Mayo team. They also did an MRI and a variety of other tests (as they do) and it was found that Chris also has spinal compression (again) at the C1 level. (Decompression at the C1 was his very first surgery when he was 2 - also with this team at Mayo - led by Dr. Stans.) The C1 is at the very top of the spine - any issues with the spinal cord at this level controls everything from movement to breathing. Since these are very complicated surgeries and Chris' body is very "unique" Mayo worked for months to build 3D models of Chris. They used these models to determine the best method of surgery. (Very cool!) We kept saying - no problem - we'd rather have them experiment on the model than on Chris! The Mayo team has also consulted with the DuPont team...so we are confident that Chris will have the very best outcome.
The spinal decompression involves removing of bone around the spinal cord. This will weaken the spine and increase the risk of injury. To remedy this, there will be a spinal fusion from Chris' skull to about his mid back. Spinal fusion is permanent. Once these surgeries are behind us and Chris is well healed - he hopes to then continue his pursuit of straightening his legs so he can once again walk without the aid of crutches.