Sunday Night: During the last "medical shift" of the day when I have to prep and load all of Sam's food and meds for the overnight period we had a little problem. He now eats 19 hours a day but meds go in 3 times a day or some just as needed. When I had almost everything in I went to load the last crushed medicine and it got stuck. When it gets blocked up you have to pull back on the syringe and push forward a few times in hopes it will dislodge the block and allow it to flush.
Try as I might I could not get the syringe to budge. A trip to interventional radiology in Pensacola for the 3rd time this month was not in my plan for this busy week. So I called Jason to come give it a try. Mind you, he is rather nervous about administering meds. Jason has more strength in his little finger than I have in my whole body and he sometimes makes me think of Baby Huey. If you recall, that was the book about the huge baby that could unintentionally take out whole forests with the swing of his little rattle. So next thing I know he has barely pushed the syringe in and water is squirting out all over us. He blew a hole in the GJ tube with the pressure. UGGGH!! ..and still the clog did not budge.
It was one of those pure stomp your feet and yell "Dang it!!!" moments. Luckily it did not blow the J channel of his port, just the G side which goes to the stomach where we put meds, not food right now. So his food was able to continue thru the night and I had to plan to go to pensacola the next day.
Monday: Well..it worked out in my favor that Bella was leaving a little earlier that morning since she is now officially a......BIG GIRL BUS RIDER!! WOW - What a big step! The bus gets all the kids in our neighborhood at one stop near our church so I still have to drive her there but that is better than a drive across town. I was crying and everything. She acted like it was as big a deal as going to Disney to get on that bus.
After that emotional send-off, I loaded up Sam and all his 10,000 pieces of equipment meds and junk..to make the tense drive to Pensacola. I sat in a waiting room for hours. During the GJ replacement procedure his BP went way up there and they made us stay for observation for a little while afterwords. The nurse said it was no wonder since they gave him no pain meds while they traced the intestines with a wire placing the thing in there. Talk about feeling crampy and miserable.
While I waited I got a call from Angel Flight saying the weather was not so good for securing a pilot for our next day's long awaited trip to Atlanta to meet with the mito specialist.. so I spent the whole drive home worrying about whether Sam could handle us trying to drive it ourselves. The whole reason for the Angel Flight is that Sam has always shown his true colors (no pun intended) on the second half of long trips back and forth from Gainesville. Well by that night I decided we could not hold off any longer since the info might be important in caring for him now before the next big health crisis. The nurse who had helped before the nursing care was suspended offered to go with us out of the goodness of her heart and I was so thankful. Not only is she a pancreatic cancer survivor but also was a missionary in Africa for 7 years..quite a life. She has taught me alot since she drifted out of the clouds to my door step. I just wish we could find a way to keep her.
Tuesday: Long drive through Alabama full of internal tension but thankful not to be alone..pretty scenes and old barns and lots of time to think.
You know me..lots of chatter all the way. I think the nurse kept me talking so I would not focus too much on what they might say to me the next day at the appt. The trip that should have taken 6 hours took us 8 and half because not only did we keep stopping for Sam and to do his meds but we also got lost in Atlanta. It was a dark, rainy night and I was pretty scared but thankfully my good friend talked me through the last leg of it over the phone. Thank you, Jenni.
A warm bed in the Dunwoody Ronald Mcdonald House and a shower made all the difference in loosening up my stiff neck. But..then I could not sleep..so many worries and questions and concerns. Sam decided to have some Brady episodes and I was up with that all hours of the night. I finally slept and the nurse took over in the early am letting me sleep in..but then that was stressful because I awoke with a start.."OH NO! We can't be late!" What the nurse did not realize in wanting me to rest is how involved a check out from RMH can be in a rush. We made it out though and with a few moments to spare.
Wednesday: We had our long awaited appt with Dr. S, the mito specialist. I had so many questions. They had alot of info to share and overall I was glad they did not leave me completely in gloom and doom. They had some positive plans and talked alot about how crucial it would be that Sam avoid any stress. We talked about ports, vaccines, TPN and meds and his weight which has somewhat stagnated for the last 4 months. They said we need to look at him like he was a micropreemie even though he was full term 8 lbs. We did not discuss much about some of the decisions made about his status in utero or how he was handled post birth which have long concerned us about whether some aspects of his status could have been avoided. I think they still don't fully understand how these decisions may have effected him and we may never fully know.
They described the little disorganized unstable energy factory in each of his cells. They said it is like a team huddle for a football game. His team is not all tight and close together following the coaches play instructions. One guy is on his cell phone..one is counting clouds and another is chatting with a friend. So..the instructions get lost and the play is disorganized. I love visuals like this. They don't know if his issues are a secondary or primary problem and they have not located a genetic cause. Mitochondria can be damaged through a chain of events or as a result of spontaneous mutation or following other conditions. They can be inherited from either exclusively mom (now known to be more rare) or from both mom and dad or not inherited at all. He was negative for all the genes they have mapped for mitochondrial disease. There is sooo much they are still trying to understand though about this disease. They do know what it means for him on a very fundamental level. His organs need optimized energy production to function well and as of right now we have these periods where on many levels he is doing just ok and then some areas always seem a bit over taxed but all it takes is a little cold or fever or being overtired or hot or just in general not up on his baseline and he is thrown over the edge which leads to systematic shut down. So it is our job to work on finding the healthy middle for our delicate little man.
Each time he is put through a major crash as I call it..he takes steps backward and has to work harder to regain them. He is in prime time for growth and development which requires alot of energy so this is an incredibly difficult time for mito kids. I asked if they expected him to make it through it and they said the chances of death in kids during this phase are less than we think. That is encouraging but as there have been many times I feel we shaved it close to that cliff I still can't completely let it out of my mind. It is always there..the elephant in the room. But God needs us to turn our focus away from worry and be the best advocates for our son's growth. He is such a fighter.
One interesting comparison that they made was to heat stroke that kills people in the summer. In those circumstances of extreme heat and dehydration, even otherwise healthy people can die from systematic organ failure. This is considered death by acute mitochondrial failure. This can happen in situations where people have top working mitochondria. With Sam having unstable dysfunctioning mitochondria, his body does not require the stress to involve extreme temperatures.His body reacts similarly to less extreme forces. It was an informative meeting and there are alot of things we can do to optimize his day to day. One of the biggest discussions was about coordination of care among all his many specialists. They want to be much more involved than I had thought but they will not coordinate everything from Atlanta. We will have to travel there every 3 months or so and call at every dip along the way. I get the impression we are part of all their research. This guy has only written 88 medical journals and has 6 drugs before the FDA for approval. They talked with me about the importance that our pediatrician coordinate his care and not leave us flandering to do it for oursleves (which is somewhat how things seem to go around here). This is common among mito kids and having a central team leader is crucial.. they don't have to be experts on mitochondrial disease but have to be willing to learn. I am hoping our wonderful pediatricians are like minded in this plan.
Late Wednesday: Long drive home and only one stop for feeling lost (thankful for the many people who came to my aid as I stood looking confused holding my map in the montgomery mcds)..and only one stop for Sam having an episode towards the end. A happy homecoming with the girls and Jason. I am soooo thankful that Sam made the trip rather uneventfully. I am also most thankful for my mother who not only has always come over and helped with the girls when I am away with Sam, but also has made it her personal daily mission since our last hospitalization to help me get the house back in shape and organized. I am sometimes in a full stressy mode and can't string together one task around here to the next. She swoops in and has been pulling together my loose ends. You never saw someone so excited to tackle cleaning my laudry room. I am blessed by her aid.
Thursday: Bella was super excited to find out she would be star of the week in her kindergarten class next week and so we worked on her "all about me" poster shown below. She gets to bring her favorite snack and do a show and tell and I am hoping to find a way to go in and read her favorite book to the class.
I am always very thankful for the many kind cards that come with prayer wishes and the love of many friends. Two came thursday afternoon during a lower moment and there are no words to say how much they meant in building me back up again.
Friday: Sofie had pajama day at school and had a great time eating breakfast on her sleeping bag. It is good to see them smiling over these fun experiences.
Prayers on my mind: My dear friend's father and their family as they make difficult health decisions. Financial and insurance concerns relating to all Sam's medical weighing heavy right now. Praying about an upcoming transition for my sister with a move to Belgium ahead and a husband deployed for the past year. Prayers of thanks in that they were able to sell their current home. Praying for a family that lost a pregnancy of twins right before an overseas deployment recently. Prayers of hope for our little boy that he not only overcome his challenges and enjoy a period without struggle to be able to focus on making strides in his growth but also thanks that he continues to show us everyday the power of God's love and the promises He has made for us all.
Thats great that you get to see Dr. S in Atlanta (not so great that you even have a reason to see him in the first place, though!)... from all I have read, he is the best mito doc in the nation (maybe even in the world?). We have wanted to bring Abigail to him, but living in the pacific northwest, that seems impossible. We have a pretty great mito doc here, but feel like it'd be great to see the BEST.. ya know?
ReplyDeleteMy prayers are with you!