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.

On our way

It’s Tuesday and we are on our way to Stanford. With the four of us going, we decided to take Big Red, our suburban. Right now Sierra is driving; she is 15 and has her permit!  Look out Calif drivers!  Stacy is in the passenger seat keeping her between the lines and Gage and I are in the back chillin’ out.

Calif sent us the addenda for tomorrow:  8:30 Gage’s pacemaker adjustment, 9:30 psych, 10:30 echoes and EKG for both kids, 11:30 meeting with Doctors and transplant coordinator, 2:00 nutritionist, 2:30 Mary Burge /social worker, 3:30 to the RMH for an interview with Keith Morrison.  Stacy and I have made a pretty good list of questions of ask.  I will share what learn as we learn it.  Sierra has continued to loose weight, she now around 90 pounds for a total loss of 15 lbs. YES, that is one of the many questions we have for Calif. I am sure they will make med adjustments as they look at her labs, kidney function, heart function etc.

Tax season is in full swing, and I have a lot of work, but there are just something’s I just have to do. I will get the work done – on time, but not right now.  Check in later.

(4 hours later) So we picked up some hitchhikers in Burns Junction. Might as well, right?  It was my sister Garrity, and her cute little boy John. They drove down from their home in Prinston Oregon to see us as we were passing by.   They were not planning on coming with us, just decided to do it. We are excited to add them to our adventure.   Hopefully Seth will understand. Later

Wednesday morning.  I took over driving from Reno to Palo Alto. Sierra (or her parents) are not ready for driving over the mountains and into the ‘jungle’.  Now we are at the Blacks in Palo Alto. Gayle, Jed, Abby, and Isabell are very nice in allowing us to stay here. Last night Garrity needed some diapers and supplies since she didn’t bring any. So Gayle let us borrow there Tesla to drive up town.  If you EVER have the opportunity to drive a Tesla, DO IT. It is an electric car that goes from 0-60 in about 4 seconds. It was a huge rush.

This morning, Stacy and I went on a nice run over to see the construction over at the RMH and then through the Stanford campus.  Now we are getting ready for the day.  Had a good pep talk with Sierra about Janice Hayhurst.  Janice is one of my heroes that has fought through may hardships in her life.  Many times she was told she won’t or shouldn’t survive, but somehow she keeps pushing.  She is someone I have looked up to for years.

First up is Gage’s pacemaker at 8:30….   

Talked to Calif

This last week Stacy talked to Hollander, Mary Burge, and other Calif people. 

On February 11th, Stacy and I are going with Sierra to Stanford for the pre-transplant work up. They are hoping to get her on the list shortly after that visit. They will post her as a Status 2 meaning we can wait at home, as long as she stays stable. The new medication they put her on is helping. She has lost almost 10 lbs of fluid and feeling better. We will take Gage with us to adjust his pace maker.

We have lots of questions about the waiting process, all of which we will discuss with them.  

After several calls to our insurance, they will cover a life flight to Calif once the 'call' comes. 

Sierra doesn't like to talk about it.  She feels good and wants to stay here. 

Here are good pictures of the kids.

 Lindsey and her awesome teacher, Dana Marlia.

 Hunter playing point guard.

Lindsey and Ayla helping Stacy with in the kitchen.

Not on the list yet.

Fortunately or unfortunately we don't have much to report.

Calif hasn't gotten back to us regarding Sierra going on the transplant list. We understand there are really sick kids at LPCH and Sierra is NOT one of them, thank goodness.  They did have us pretty worked up about Sierra's heart conditions, and the need to go back on the list, and they needed to visit with the heart team to confirm the appropriate action.  It's been a week and a half now. We are going to call tomorrow and review our questions with them and see where we are at.  It would be great if she did not need to go back on the list, and maybe that is what they will decide.  We just need to visit with them.

With the addition of this new med, Sierra's puffiness and water weight has stayed down. She has lost about 7 lbs of fluid, and looks and acts good.  So we are not feeling an urgency to get her on the list. But, if it's going to happen, let's get 'r going before issues arise. Sierra is on a mountain of meds so who knows how much wiggle room we have.

Having said all this, she and the rest of the gang are doing great.  Days are full with school, basketball practices and games, dance practice, skiing, work, church, and living. All stuff we love.
  

Understanding the zeros

I know this post is a bit overdue. This is all something that is hard to face. We just want to go about our lives and pretend none of this is going on. But it is, so lets deal with it.  To clarify some of the issues.
Both Sierra and Lindsey did get zeros. That is awesome!  Lindsey does not need to be seen by the doctors for 6 months (a luxury Sierra has never had).  Lindsey is actually bummed about that, she likes going to Calif to see her friends, teachers, nurses, and doctors.
For Sierra, the zero is both good and not-so-good. It is good because she has no rejection.  The body is not fighting against the heart. That is great!  It is not-so-good because the heart failure can not be blamed on rejection.  If it was rejection that was causing these high heart pressures and poor coronary arteries, then they would have something to treat.  IV's and meds could get the rejection under control. But since there is no rejection, there is nothing to 'treat'. The water pill they added has seemed to help.  She has lost about 5 pounds of fluid since last week.  This water pill is in addition to all other meds she is on. The MOST important thing to notice is how she feels.  Right now she feels and acts good, she eats well, and her spirits are up. I would like her to try exercising a bit but who knows how that will go.  She really hates to talk about it.  She just wants this all to go away!!
We haven't heard from Calif regarding going back on to the list.  They were going to get back to us Friday, but still haven't heard anything yet.  We figure they will let us know when they have to an agreement on the best treatment plan.  I have many questions to ask and understand.
Other than that, it is life as usual.  Lindsey had a basketball game, Hunter had cub scouts, Megan had practice, Sierra had dance practice, and Gage loves to tag along. We are involved in skiing, building snow men, and anything else we can find or need to do.  Other than talking about this on our blog, life just keeps matching on.  The best thing we can do is just live life and not wait for something to happen.
Till next time,

Treats with the cousins

Hunter and cub scouts at the police department

 Once there was a Snow man...

 Tall, tall, tall....

 In the sun he melted,...

 melted....

And great was the fall there of!

Poor guy didn't even last 12 hours.

Jan 14, Biopsy results, Round 3??

Had the biopsies today, and as Stacy and I had worried, the news was less than great for Sierra.

To help set the stage, Dr Perry does the biopsies, and has done for over 8 years now.  He does an excellent job, and we are very comfortable with him and his care, but he is a man of few words.  We have learned that if everything is OK on the biopsy, you might get a 'she looks fine'. But if there are concerns he will talk.  This time he 'talked'.  He told Stacy that Sierra's heart pressures are up (a sign her heart is not beating properly), her coronary arteries are slightly more restrictive, and heart is squeezing hard enough.  This helps explain the puffiness, fatigue, nausea, and the cough.  He talked to her about listing her on the transplant list. Something we were fearing.  I'm just glad we will have options.  
Stacy says it best in her Facebook posting:

Just to set the record straight. Sierra is NOT on the transplant list. They are having a meeting Friday and would like to list her as soon as possible. She is stable and doing fine. The wait is so long now that they want to get her on the list before she is really sick as transplanted hearts do not respond well to VADS (something like the berlin that Lindsey was on). Her Pressures in her heart were high today and with her swelling she's been having, she is not getting better. We will know more Friday. She will probably be listed as a 2 and we may be able to wait at home as long as she is stable.

That is really all we know for now. Unfortunately we know to well what is ahead of us.  Still many questions to understand or at least ask.  Mostly what new issues will arise from a re-transplant, antibodies, kidneys, rejections, etc.  Are there new therapies developed in the last 2 years we don't know about. How have other patients responded to re-transplant. What are the VAD options? Will our insurance allow us to wait at home? How fast am I allowed to drive to California once we get the call :) When or how should I start growing my facial hair. And the list goes on.....

For now, they are coming home . Going back to school, skiing, dance, life, the way it was and should be.  Sierra will be adding another med to help with the fluid retention, but other than that, everything else is the same.

Oh yah, Lindsey's biopsy went GREAT! Everything was normal. Rejection results for both the kids tomorrow. Depending on final results of the biopsies, Lindsey could go 6 months before her next biopsy. crazy.

Sure fled bad calling my mother today. This kind of news is hard on her. She is a wonderful Mom and grandmother to my kids.

Here are few fun pictures so far from the trip. 

Waiting at the airport.

Who can forget Shannon.