Family

 This has been a month of family! We are LOVING having Mimi and PopPop live so close and see them most days. Maddy has finished 6 weeks of First Grade and is doing a great job for me.  She continues to participate in Hippotherapy, but we are otherwise still quarantining pretty strictly because of the kids immune deficiency. The kids have been spending a lot of time together playing games and watching movies. Maddy had her first sleepover at Mimi's last night. And we snuck away with Ellie for ice cream one day! Bruce has recovered extremely well from his surgery. He meets with Oncology tomorrow to determine next steps. We are so grateful to everyone who has supported us in so many ways. I started my leave from work last week and that has helped my stress level as I have more hours in the day to meet the needs of our family. I am still extremely busy since we have no nursing help right now with the kids and their medical care is pretty intensive on a daily basis. We are just using this time to focus on family and loving each other well. 

Here are a few highlights from the past two weeks:

As for a medical Update:

We had three appointments with our Houston team this week via Telehealth. The first was with a nephrologist to try and sort out the changes we have been seeing with Alex's blood pressures as well as fluid balance. At the end of the day, it comes down to Mito. His kidney's are not functioning correctly because they simply lack the energy needed to do their job. Our hope is that this will improve and is because of the stress his body was under with surgery and recovery. 

Our second appointment was with Dr. Koenig, our Mito specialist/neurologist. We did not make changes but are watching/need to follow-up on a few things with each kiddo. For Alex, the main thing is we need to get a spine MRI to check on his syrinx. (Fluid in his spinal cord) Alex's syrinx is extremely large and any change could cause damage to his cord. Other than that it is a matter of not stressing his body and trying to let his recovery continue. For Maddy, she has been having headaches that Dr. Koenig believes are migraines based on Maddy's answers to questions and my input on what she tells me. At this point we are going to just watch as we don't want to add a daily medication unless absolutely necessary. She is also having some peripheral neuropathy. We are taking the same approach in this area. Dr. Koenig did want us to follow-up with the knee surgeon sooner rather than later because her knee is starting to give out on her. (We actually got an appt for this week). Her biggest concern is that we get a echo of her heart ASAP because past testing plus some of her other issues put her at very high risk for cardiomyopathy. That was the gist of our appt with Dr. K.

Wednesday we met with Dr. Pacheco, our immunologist. From an immune standpoint the kids are doing really well. (that's what happens when you stay in your house for 5 months 😉) She wanted to be sure we were taking all precautions to keep our family safe. Dr. Pacheco is a doctor that has watched Alex fight for his life many times. She knows him better than almost any other doctor. When Alex let her know that we are struggling to come up with a plan to control his pain adequately, she was adamant that "pain is not an option". I have reached out to our team here once again to try and come up with a plan that is acceptable with everyone, but if we are not able to we will work with Houston on this part of Alex's care. Alex was so relieved to have a doctor validate that he shouldn't have to hurt and or live with anxiety of worsening pain with no medications to help. He was actually in tears by the end of his appointment because this has been such a concern. 

While having a team 5 hours away is not always ideal, having doctors that have walked this road with us for 13 years and understand all the facets of Mitochondrial Disease is so important in the kids care.

Please continue to pray for us as we navigate the next few weeks with decisions to be made in treatment for Bruce, Alex and Maddy.

Ali