Counting Our Blessings

Counting Our Blessings

Sunday, September 30, 2018

Mayo Update

As I reflect back on our week in Minnesota I’m grateful for so many things.  Grateful for the Lord's provision in making this trip a reality for us. Grateful for so many peoples prayers and support. Grateful for visits with long time friends and Grateful for the opportunity to have so many appointments and tests with incredible doctors all in a weeks time.

I had heard many incredible things about the Mayo Clinic but it more than surpassed our expectations.  It is an incredible place.  I have never experienced anything quite like it.  What do I mean?  Well for starters every doctor we met with spent over an hour with us and it was clear that they studied our files and talked with other doctors at the Mayo clinic about our case.  They have a team approach.  They all work on Salary so they have the gift of time.  Everything is done onsite.  Every test, lab, research etc.  In CA we see doctors and specialist all over town.  Every procedure is scheduled weeks out, and different companies have to mail things out to you.  At mayo we were able to do tests, procedures, labs, x-rays, and many doctor visits all within a week.  Some tests that the doctors wanted after we saw them we either did same day or next day.  I joked with Jason that everyone of the staff must take Happy pills.  Everyone seems to love their job and treated you like you were very important to them.  Everything is run so efficiently and set up super well  even for people who have disabilities.  They have special needs restrooms available on every floor, the check in Kiosks you can move down to wheelchair level, the drinking fountains are normal and wheelchair height and have dixie cups for those that can’t drink from the fountain.  They really thought things through.  People come from all over the world come get care from Mayo. If you want to know more Ken Burns did a documentary that aired on PBS this week.  You can go to PBS.com to watch it. After my first appointment I downloaded the Mayo Clinic app on my phone.  This gave me access to my schedule, labs, test results, and comprehensive doctors notes and records(they take amazing transcribed notes after each visit).  All of these things are downloaded on my app within an hour of completion.  I was able to see my x-rays, blood test results, doctors notes etc.  I have had other portals here in CA but nothing is ever loaded and the doctors don’t put any notes from your 10 min visit.  The place is huge and I can’t say enough great things.

Our main reason for this visit was to see Dr. Selcen.  A doctor who has the most expertise with our specific rare disease.  When we first met Dr. Selcen we got the impression that she was just as excited to see us.  She has a passion for studying Myofibrillar Myopathies. If you look up Myofibrillar Myopathies you will see her name all over many of the publications. She is a research doctor and sees other patients with our particular Myopathy.  She is brilliant and every doctor we encountered talked about how brilliant she is.  She spent close to two hours with us at our first appointment and we met with her a total of three times.  She explained what was going on with the genes to cause this Desmin protein to aggravate the muscle fibers.  We felt like we gained a ton of understanding and felt hopeful that she is working hard to find a way to help this Myopathy.  She gave us some handouts on some current studies that are being done.  She recommended a couple antioxidant supplements.  They have done some testing and have found that one may help to slow down the aggregation of the protein in the muscle that is causing the weakness and the other helps keep the muscles that haven’t been effected energized.  They are over the counter and is no way a cure but they have found it to help.  We are looking forward to giving them a try and keeping in contact with her as more questions and symptoms arise.  She is a part of a Desmin Facebook page that she invited me to.  It’s a place to connect, share symptoms, hear about Dr. Selcens research and advances.  I told her I was passionate about helping to educate others especially doctors so they can help others that may be struggling to find a diagnosis.  She had Noah and I take pictures and video for educational purposes.  So thankful to have met her.

Another reason for our visit was to get some clarity and direction on Noah’s heart condition.  This was another positive experience.  We met with a Pediatric Cardiac transplant doctor named Dr. Johnson. This doctor knew Both Dr. Chang and Dr. Alejos our current doctors here at CHOC and UCLA.  He had wonderful things to say about them both.  We love our doctors but have felt unsettled on the fact that they do not agree with one another and it has made us question if we are doing everything wended to be doing.  Noah came into this appointment really wanting to understand his heart condition.  He left feeling like this was the first doctor to really explain everything to him.  Dr. Johnson explained to us that most people with Restrictive Cardiomyopathy do not survive past two years and that most of them do not even make it to transplant.  This is why they put them on the Transplant list right away.  This is why Dr. Chang has been concerned with him being off the list.  Dr. Johnson agreed that his EKG is one of the scariest he has seen.  However his Echo looks amazing and doesn’t make since with his EKG.  His pressures and heart looks good for someone who has RCM.  Since his EKG has been the same since his first one at 9 years of age you have to assume that its because of his muscle disease.  Dr. Johnson agreed with UCLA to keep monitoring him every three to six months and if his echo starts to look bad to list him immediately.  We asked him if he has ever seen anyone get better with RCM and he said NEVER.  Dr. Selcen brought up the point that it will be better to be transplanted  before his muscles get weaker.  This is something we will have to consider in the future.  We came to the realization that it will be a decision that Noah will have to make as he will be an adult in just a couple years.  He understand this and I think its one of the reasons he really wanted to understand his condition.  We left this appointment  feeling more at peace about the decision to keep him off the list for now and feel more confident in our doctors at UCLA. 

My visit with the pulmonologist was difficult.  She was a wonderful doctor and really helpful in explaining how my breathing muscles are being effected by my disease.  It was disheartening to see such a big decline in a year and a half.  She talked through the tough things ahead like trachs, and 24 hour ventilation. These things could be far down the road but it seems like we get closer all the time.  It’s difficult to hear the hard things but also good to prepare our hearts and minds.  My visit with the cardiologist was good.  My echo looks really good.  Even though I’m stilling having v-tach episodes, the fact that my ICD hasn’t gone off is good.  If it starts to go off then there are other meds and treatments that can be done so we felt hopeful.


This is really long so I will end with a heartfelt thank you.  Thank you to those that our invested in our story and care enough to walk along side us. Thank you for all your prayers and words of encouragement.  We felt so lifted up by all of you this last week.  

2 Corinthian 4:16-17
So we do not lose heart, though our outer self is wasting away, our inner self is being renewed day by day.  For this light momentary affliction is preparing for us an eternal weight of glory beyond all comparison.

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