Thursday Update, Meeting with Doctors

What a few days!  We are now driving through Nevada on our way home.  Yesterday (Wednesday), we had appointments with doctors and staff from 8:30 until about 4:00. Gage and Sierra both had echoes done. Gage received his pacemaker adjustment.  He now has his 24 hour halter monitor on to see if they have the adjustments correct. Pending no more issues, they will see him in June or July, depending on the other kids. 

Asked one of the questions I always dread with Gage. Is his Cardiomyopothy still progressing?  He is acting so well I just assume his heart is doing okay.  Dr. Rosenthal said that when he checked his echoes he is surprised NOT to see a normal heart. If fact, his echoes looks worse over the last 18 months. I was really hoping he was holding his own…but I guess not. There is nothing we are going to change with his regiment, checkups, meds, or supplements, just something we need to watch and prepare for.  No real timeline on if and when he will need a transplant.  Best thing we can do is keep pushing the calories, he is very thin for is age and height.

But that was not the main focus of our trip; that was more of a side note to the main events.  Had a LONG visit with Dr. Seth Hollander and Dr. Rosenthal about Sierra and here is where we are at. She has coronary artery disease and needs to go back on the transplant list.  The heart squeeze is okay, but it is not relaxing properly between beats. They can see this from the increased heart pressures during her biopsies.  Because of the many years of rejection, both cellular and antibody, the coronary arteries have begun to shrink which cause the increased heart pressures and stiffening. The coronary arteries are a part of the donor heart and are the arteries that wrap around and feed the heart. Right now they do not have good therapies to fix this problem, only retransplant.  They said this is common with transplant patients, especially those with a history of antibody and cellular rejection. Right now she does NOT have rejection, but the damage has been done.

Okay, so now what. She is acting great and the new med has pulled 15 pounds of water weight off.  They want to get her on the list now knowing the wait is well over a year.  The hope is she can wait at home and get life flighted to Calif when the ‘call’ comes. We have some homework to do regarding an air ambulance.  We need to be able to go from the ‘call’ to LPCH in about 3-4 hours, even though when we get there, she could wait another 12+ hours before surgery.  That is the best case scenario.

I asked the big boys about the worst case scenario, just to prepare myself.  They said the progress of the disease goes slow, then accelerates towards the end.  Basically the same way Sierra and Lindsey’s heart failure went years ago; starts slow, then crashes.  Then my next question was regarding VADS or other mechanical support (like the Berlin Heart) during the ‘crash’ period. They said as of now, they are NOT having good success with VADS, because the mechanical support assist with the squeeze of the heart NOT the relaxing.  Milrinone and other IV treatments have helped prolong the wait, but don’t count on the VADS.  Sooo, we do NOT have that crutch to lean on if/when the time comes.  

This brings us full circle.  The reason they want her on the list now, when she is feeling good and the large amount of meds are working, is to get her to transplant BEFORE she crashes.  If she crashes, and the meds are not working there is NO mechanical support to lean on. This means we lose her….

They showed us some statistics regarding transplants, about 70% survive, 30% don’t, mostly due to this coronary artery disease.   Nothing is guaranteed is it?

So now the wait begins, the doctors and other heart staff meet on Friday for the final determination and listing. She will be listed as a status 2, meaning low priority. We will ALWAYS have our phones with us with alternate numbers for grandparents, office, and other relatives.  If we travel, we will need to check with our air ambulance service, or be suspended from the list until we get back.  Most likely, we spend most of our vacations, holidays, and reunions in the good ole’ Baker valley area.

Sierra was there for all these conversations.  She says she is ready to face this head on.  She especially feels bad for Nicholas and his family.  She has felt an attachment to him for the gift he gave her.  She feels horrible or even guilty that her body has rejected his heart to the point of needing another transplant.  She wishes she could keep Nicholas’ heart, and thank him someday in the afterlife.  She has wept more about that, then anything else.  I sure love that child.

After our appointments, Stacy and I went to the RMH to talk about our day with Keith Morrison and the crew. They want to update our story on Dateline sometime in May, but this all depends on where ‘our’ story goes.

 Now here we are, somewhere still on Hwy 95 in Nevada. Gage and Sierra are doing homework. Garrity is driving, and Stacy is telling cool nursing stories. No one wants to hear my cool tax accounting stories, go figure. Our plan is to go back to normal life, school, church, work, basketball, dancing, and everything else that goes with raising 5 kids. I fully believe we will be able to wait in Oregon the entire time. Stacy believes it won’t be that easy, and complications will evolve.  Who knows, maybe Gage and Sierra will someday be waiting side-by-side in the hospital. If it comes to that, we will deal with it.  

Thanks Garrity and John for tagging along.  They were great to travel with and help with the kids.

We had lunch with Kade and Stephanie Thomas from Adrian.  He as quit his job back home and has move to Calif with this family. He will work for an electric motor shop in the bay area as they continue to wait for Tristin’s transplant. They have been waiting since September and are doing well.

We saw Dr Axelrod in the hall way. He wanted to know how Axelrod, our dog is doing. I gave him a good report on the family dog. 

Happier and shorter posts in the future, but for now, I needed to get this off my chest before I head to the office.

By the way, happy 2 year anniversary Lindsey, today 2 years ago we waited to go into surgery. She is doing great.

Dr Axelrod and Dr. Wright swinging by to say hi. 

   Blood draws needed. 

Hours before going in 2 years ago

Hours after surgery 2 years ago.