How far

I follow about a dozen or so blogs and Facebook pages of other kiddos with complex medical problems. Most of them written by their mommies. I identify with many of these moms even though our children have different needs. I find that we all write about similar experiences but each with their own unique spin. Each child different but often a path very similar.

We live our lives for our children and do anything we can to improve their quality of life. We fiercely advocate for them as they cannot do for themselves. We are responsible for protecting them. With a future full of so many unknowns, we live day to day hoping the worst case scenario never happens.

Recently I have been focused on what could someday be a worst case scenario for Logan and I am having a hard time wrapping my head around it. I am so fearful of him becoming sick. I worry that one respiratory infection could throw him into a tailspin that he may not be able to fight. 

We know that part of Logan's brain is degenerating. We have no clue what this means for his future. We are being told that he will probably continue to lose skills and have a harder time fighting infections as he becomes weaker. I worry that he will land in the ICU again and we will have to decide how far to go. How hard will we push to get him healthy and at what cost. 

I have been waging this inner battle of how far is too far. What is necessary and what is cruel. We live in a world full of life saving technology that can bring a person from the edge and back. As a nurse, I know these stories all too well. And with children, their resiliency combined with top notch medical care can often provide miraculous results. But I know too well the other side of these life saving measures. 

Joel and I have consented to life saving measures for our son and I actually acted out these measures on him myself when he stopped breathing during a seizure. We have put Logan through procedures such as a feeding tube and tracheostomy tube in order to prolong his life and improve his quality of life. But at what cost? He has to have a feeding pump connected to him by tubing almost all day, every day. He has an uncomfortable breathing tube in his neck that requires intermittent suctioning to keep his airway clear so that he can breathe. I could go on for pages telling you the downsides of what comes with these medical interventions. But without them we would not have Logan. 

But guess what, regardless of what we do for him, there will be a day when we don't have him. I just hope that when that day comes, I do not regret the choices we have made for him. I want to look back and know that I was a fierce mommy that gave it my all whether it be making the choice to consent for further intervention or making the choice to let go and give it to god. So far, I do not regret our path. I know that every decision Joel and I have made is done with careful consideration of all of the options. 

I am so proud to be part of a community of other mommies that are fierce protectors of their babies. I am not alone in this and knowing that, provides comfort when the fears of the unknown creep in. Thank you to all the mommies out there that share your stories whether it be by blog, Facebook, or day to day conversation. 

Summer Fun

Last week Grandma Cindy, Aunt Jeni, and Cousin Ty came down for a visit. We celebrated Cousin Taylor's Birthday and went to the Children's Museum. Please enjoy the photos!

Tube troubles

Last night Logan vomited pediasure. This is generally a sign that his feeding tube is out of place. I wasn't sure if the tube was perforated or out of place as we are giving him new vitamins that do not dissolve in water and have caused some close calls with tube clogging.

After starving him most of the day we got the last appointment for a feeding tube change this afternoon. My mother in law traveled with and was a great nursing assistant! Actually, she is a darn good nurse herself. 

We laid him on the radiology table and the first picture showed his tube was coiled in his stomach. The tube should go into his stomach and then travel and stop in the jejunum. 

For a routine change, the radiologist puts a wire through the old tube, pulls it out, and then threads the new tube over the wire back into place. A picture is taken with contrast material to confirm placement.

Today the tube had to be removed and the radiologist had to start from scratch. This takes a little longer and isn't the end of the world but he had to do it a few times as the placement was not ideal. The last attempt worked and we are hoping it will stay in place a while. 

Now he is tuckered out and hungry! Feeding is going and two naps are complete. Now he is ready to party!

Funk

I'm in a funk. Changes keep coming and I don't seem to have the energy or interest to keep up. I find my to-do list growing and I seem to find other tasks to complete in order to divert my attention. Summer is flying by and there is a ton to do. Yikes! 

As for Logan, he is doing well. He is healthy and continues to grow. He has been in the pool, to the park, to a play, and most recently his cousins ball game. His wheelchair has put on some miles this summer!

We have started him on a new vitamin/supplement therapy. The Mayo neurologist and Logan's primary neurologist have recommended this therapy. They cited a recent study that used biotin and thiamine and showed improved outcomes in patients with similar symptoms and MRI findings as Logan. He is on a large dose of biotin, 40mg three times per day. This means we crush eight tablets three times a day, put in water, and give through his feeding tube. We then say a prayer that it does not clog. He only gets one tab of thiamine three times a day. I am not sure what we should be looking for. The study I read talked about this therapy decreasing or preventing crises. I would like the therapy to lessen his daily issues with movements, vision, and overall development. Is it too much to ask, just a little miracle, please?

So into the unknown we go! If anyone has any knowledge of a regimen like this and can tell me where to get a larger dose per tab or a liquid form of the medicine please leave a comment. 

I have also received a letter from his Mayo Clinic neurologist summing up our visit. She stated in her note that she believes he has a probable mitochondrial disorder. When his Exome sequencing is back in October, we will then pursue more mitochondrial testing. I say this without knowing the results of his Exome sequencing as I feel it will not be the answer to our prayers. With the information we were given during our Mayo visit I can't see why this isn't mitochondrial. 

He has a documented mitochondrial mutation that sits very close to another mitochondrial disease called Leigh disease and his symptoms are classic for this disease except for his MRI findings which are not classic for Leigh disease. It makes sense that he shares a lot of similarities with this disease if he truly has a mitochondrial defect where the testing has identified. 

My gut has always pulled me toward mito as the culprit of Logan's medical and developmental problems. Now we wait and see what our current testing shows. With Logan, the hunt for a diagnosis has proven to be a wild goose chase. Hopefully we will catch the goose or maybe a golden egg soon!

Bubba took a snooze on the couch while I wrote this post. Seems that the all star game put him to sleep!

Bubba and Daddy 

Keeping cool in the pool. Logan seems to enjoy the water, his movements slow down! 

Brother and sister watching cartoons. Too cute!

Aunt Michele and Bubba hanging at the ball game.

  

New therapy

Today I received a call from Logan's neurologist office. He will be starting biotin and thiamine therapy. These are both vitamin supplements. I was really unsure of the reasons behind this and the medical secretary had no idea. When we came home, there was a letter in the mail from Mayo Clinic. We are so fortunate to have doctors that send copies of their notes to our home. 

Basically it summed up our visit with the Mayo neurologist with a few addendums related to the plan to treat Logan's movement disorder. The letter also pointed once again to the possibility of a mitochondrial disorder. Mito disorders are often treated with supplements in order to lessen symptoms and or slow the progression of the disease. Hmmm, so are we starting this new treatment with the thought that he may have a mito disorder. Well it is worth a try! 

I have spent the last hour and a half researching mitochondrial disorders, more specifically the mutation that has been found in his mito DNA and now my head is swimming. I was in this same spot last year and feel a little deja vu. I will post tomorrow with more of my thoughts as well as some fun updates on what Logan has been up to this summer! Until then...