GI appointment and update

Broc saw GI and they are very happy with how he is growing. A gentle giant I have. Well 2 including his brother. Both boys have now hit 5 feet. I will need a step ladder soon. We are still trying tropic feeds at night for 12 hours. So far he is only tolerating 72oz in total but we will take it. It’s really helping his liver. His bilirubin level is coming back down under 2 so we are very happy. Broc continues to suffer with ileus’s. We will do some imaging soon to see how his intestines look after they narrowed in March.

Since Broc has stopped taking his Co-q10 he is napping more often. Some days his naps last 3 hours which is worriesome as we never know if he’s ok or getting ill. But he wakes up fine and ready to go again. Hoping that once he has had his biopsy we can start it again. Since starting his meds for his TS he’s having a lot less tics daily. He is able to focus a little more too.

We thought that because we didn’t hear from Cardiology everything was fine with his MRI results. I got a call last week to say Brocs case is once again being presented to the Cardiology Team to see what they think. We have been told that surgery is still a small possibility. Not knowing is proving to be very difficult for me. I really like to plan and have everything organized and I’m not able to do that. We are also waiting for a date for Brocs muscle and skin biopsy.  He will be having an iron infusion in 2 weeks to give him some much needed iron.

Our Buddy got new glasses today to help him see better.

Adding a new team member.

Lots of things have been going on with Broc lately. He is getting really tired at times just from a therapy appintment or playing with his brother and just lays around watching his tablet or reads a book. He is constantly looking pale despite getting iron infusions.

Broc has been having lots of vocal and motor tics. For a long time we thought they were just regular tics that kids get from their body changing etc. After his really bad bacterial and fungal blood infections last Summer his motor tics became worse to the point that we would leave a restaurant or a gathering. When they have become excessive we have kept Broc at home to protect him. Its only natural for people to stare when someone is making weird jerky movements or talking really loudly or making a lot of noise. Sometimes they have been really scary and he can hurt himself so we have to keep a watch on him 24/7 unless he us sleeping. Last month Broc was diagnosed with Tourette Syndrome. Although it was hard to hear yet another diagnosis for him we kind of knew it was coming. It does help us understand a lot of things that Broc does and his behaviors. He was also diagnosed with OCD.

We also saw the Metabolism/ Mito team this week. What an amazing Dr. We prayed to really find a Dr that is willing to help us and also have a vested interest in Broc and his health. For Broc you really need to think outside the box. He had really done his homework before hand by reviewing his extensive chart. He also asked for my version which gave him a great perspective. We have a great plan which involves 3 steps. They will re-analyze his Whole Exome Sequencing that he had done in 2015. He will be scheduled for a muscle and skin biopsy later in the year to see how his mitochondria function and what they look like. He will also have an extensive lab workup to look at all the chemical levels in his body that have to do with his mitochondria function.

He said that although Broc does have the LTBP4 Gene mutation it really doesn't describe what is going on with his body and how it is declining every year. It could possibly play a small role but he would like to find the source of his issues. If Broc does get a Mito diagnosis it really won't change much. There is no cure or treatment for it but it will give us more tools to work with. Ultimately we want the best quality of life for Broc. The more tools we have the better.

Broc will be having his cardiac MRI and Gj tube surgery at the end of next week.  My ankle surgery is scheduled for the week after that.

We thank you for all your love,prayers and support for our family.