Heart Evaluation Continued

We recently returned from another trip to Salt Lake for evaluation, this time mostly for heart transplant. Last Monday, Sarah got scheduled for her heart catheterization to be done on Friday, August 31st. We decided to get as much done in that trip as possible, so we rescheduled her liver MRI for Thursday afternoon. I got a call on Tuesday from the heart transplant coordinator asking if we could schedule more appointments and testing the following week that would take two days. I told her we’d already be there Thursday, and we decided to just cram as much into the day as we could and then we’d only have to go down again for one more day later to finish up. So by Tuesday night, we were making arrangements to leave the next night so we could start with a fasting lab draw first thing (7:30) Thursday morning. It was pretty overwhelming, but with the kindness and understanding of flexible employers, and a wonderful mom and second daughter, we got it all arranged and left around 9:00 Wednesday night.

6:00 came so early the next morning…. We hurried and got ready and headed up to the University of Utah Medical Center for a day full of appointments and tests. At the lab, they drew 20 1/2 tubes of blood from Sarah’s little arms! (The half was because after 12, it clotted and they had to finish with another poke.) They also sent us home with a couple of other tests that can be done in the privacy of our home. With that out of the way, we checked in to the heart transplant clinic, and were immediately taken down to radiology to do an echo cardiogram. The lights were dim, and after little sleep the previous night, we succumbed. I think we all got a little nap during that one! Having had many of those in the past, Sarah was comfortable and she did great with that, too. Sarah’s morning labs were fasting ones, so we hadn’t eaten anything yet, and by 11:00 she had to be NPO (Latin term: Nil Per Os – meaning “nothing by mouth”) for her 3:00 MRI. So, we sent our daddy after bagels and yogurt at the cafeteria for us to eat during our next appointment. After the echo, we met with the social worker, the transplant coordinator, and the nutritionist. A lot of the information was similar to the things we learned about liver transplantation, but there were some huge differences.

Match: Organ matching for heart transplant requires three things: that the donor and recipient have the same blood type, the same (or at least noncompeting) antibody set, and the same body size. We don’t know yet what her blood type is or what antibodies she has that would rule out potential donors, but we do know that Sarah has a little advantage in finding a match in the size factor. Because she is a small adult, the organ pool is a little larger for her because she can use a heart from a large child, teenager or small adult. (Although size probably plays a factor as well, liver match is mostly based on blood type.) Listing: Candidates for heart transplant are listed according to blood type. If you’re an O, you get in line behind all the other O’s. The rarer A, B and AB blood types also have “lines” (which are shorter), and you just line up according to when you were listed. But, there are also different phases in each of those lines, and based on your current health, you might be phase 7 (at home, doing pretty well) or phase 2 (at home, doing OK, but struggling with heart failure symptoms). After that, as your health begins to decline, you move to phase 1B (at home or in the hospital, with therapies to keep you as healthy as possible) and finally to phase 1A (at the hospital, on life-saving therapies and often machines to keep you alive until a donor match can be made.) Not a pretty picture over all, but it’s the best way to make sure those that need it most get the hearts as they are available. This list is always changing and people are given “cuts” in their line as their health requires it. (Liver listing is based on the MELD score, and although blood type plays a role in which line you’re in, those with the highest scores get the livers first.) Time:¬†The average wait time for an O patient is about 2 years. The others can be as soon as 1 year. Heart transplant patients are often hospitalized for a while prior to their transplant to keep them stable and as healthy as possible before surgery. If not in the hospital, it would be necessary for Sarah to live in the Salt Lake area for a while before surgery so she could get there quickly if a donor match was made. (Surgeons only have 4 hours to get the heart from one patient to another…. time is of the essence.) After surgery, the average hospitalization time is two weeks. Following her release from the hospital, she would have to be in the Salt Lake area (yes, Lehi could work!) for about 6 months. So many labs and biopsies are required at first that she’d likely be at the hospital daily at first, and then slowly taper down to weekly appointments. (Liver transplant patients have to stay in the area after their release for about two months.)

With the possibility of having a heart and liver transplant, it will be interesting to see how they work out which criteria and status rules. I wonder if needing both organs would play into that equation since they would want them from the same donor, and if that would give her an opportunity to receive the second organ sooner than expected….

Following the heart appointments, we headed over to the University of Utah Medical Center for Sarah’s liver MRI. Because it was her second one, she knew what to expect, and things went pretty well. She had to have an IV (which she hates), and forgot to turn on her ipod, but other than that, things went pretty well. We headed back to our hotel with dinner and ignored everything of importance for a while. We went to bed that night very tired, and pretty overwhelmed with all the new information, concerns and questions that followed facing a likely transplant again.

All prepped and ready

The next morning, we set off to Primary Children’s for Sarah’s heart cath. (The cardiologist puts a catheter through the large artery in the right side of her neck and into her heart. Then he shoots contrast in there and watches where the heart pumps the blood and how the flow is. They can also measure the pressures in her pulmonary arteries and check for blockages and collateral vessels and document her heart’s anatomy.) The most important thing they wanted to check was her pulmonary pressures. When a kid has a fontan, it is common for the pressures in the pulmonary arteries to be fairly high. Sometimes these kids get what they call “fixed pressures” and after transplant, the lungs don’t respond well to the lower pressures of the new healthy heart, and it fails. She had to have another IV placed, but they have the coolest new thing at the children’s hospital now. It’s called “J-Tip” and is a needle-less “shot” of lidocaine pushed into the skin with compressed air. It numbs the area before they have to poke the vein. It’s so cool…worked like a dream! We decided we like the Children’s hospital better. They have J-Tip for the IVs, AND gowns that actually fit!

Settled in at Same Day Surgery, waiting to go to the cath lab

We had to wait quite a while for the actual procedure, but they moved us around enough that we felt like we were making progress. Finally, about 11:00, they took us down to the cath lab where we said goodbye and left her in good hands. She was upset and teary (procedures make her very nervous), but was so very brave. We went up to the lobby of the 3rd floor and sat and talked. I know we were getting ahead of ourselves, but Steve and I finally started talking about all the things that would need to be arranged and changed and fixed in our lives before all of this could happen. Then we talked over some of the things we’d need to¬†arrange and change and fix while it was all happening, and how in the world we would do it…. And then we talked over how we’d arrange and change and fix things after it’s over… That’s when we got a little out of control and then decided that we’d have to allow ourselves only a short time each day to think about this and then move on. We’d write notes when thoughts pop into our heads, and get our questions or concerns on paper so we wouldn’t have to think about them so much. We held hands, stared at the floor, and just tried to be “still” for a bit. The beeper they gave me went off, and we headed back down to the cath lab to talk to the doctor. He said she did great, and showed us the images they got. It was so cool to see what her mixed-up little heart is still capable of, and how very wonderfully everything was still working. He said her pressures looked really good (not fixed — yay!), and things looked much like they did 5 years ago, maybe a little better. He said everything looked great, and though the race wasn’t yet over, we’d cleared another hurdle!

Visiting while the nausea settles

Recovery was a little harder on Sarah than it’s been before. She must have had an anesthesia cocktail that didn’t really agree with her this time. She got pretty nauseous and just as we were getting her dressed to head out, she threw up and had to hang out quite a while longer before we could go home. Grandma and Grandpa Brown and Aunt Susie were passing through from the Brigham City Temple open house and stopped in to visit for a while. Sarah didn’t feel very good, but she perked up enough to talk for a bit, and the distraction was a very welcome one for all of us.

Just a bit before we were ready to go, Dr. Everitt (the heart transplant cardiologist at Primary Children’s) came to see us and talk to us about the heart cath. She was pleased with the results, except that pressures that good may mean that Sarah might not need a transplant…yet. Pulmonary pressures in a healthy heart are generally somewhere between an 8 to 10. Hers are measuring 12 right now. With fontan patients after a transplant, it’s common for the pressures to be somewhere between 10 and 12… so just about where hers are right now. So the question is, if transplant is the treatment to solve the problem of the PLE (intestinal disease) and the liver damage, and her pressures are not changed with a transplant, will it in fact impact those other organs and fix the problems? Because of the newness of this “transplant after fontan” type of treatment, there isn’t much data available. Her anatomy and these better-than-expected pressures make things more complicated, and really not knowing if it will help or not, it doesn’t really seem worth the risk at this point. So, now we’re back to really not thinking transplant will happen any time soon. Yes, emotional roller coaster ride…revisited. We’ve done this part before, haven’t we?

We still don’t know about the extent of the damage to her liver or anything about the nodules on it. Liver transplant still could take over and change things yet again.

So, after the last few days have passed, we had an amazing Fast Sunday experience focused on the Savior, His Atonement, and hope in our struggles, and instead of feeling numb, we’re feeling calm and at peace. This situation is truly in His hands, and whatever is supposed to happen for her will. He knows us and loves us, He sees the big picture, and He knows what the optimal circumstances are that will bless her the most. He has the power to do whatever needs to be done for her benefit. We just have to remember that and trust Him. It really will be okay.

~Julia

4 thoughts on “Heart Evaluation Continued

    1. thank you for sharing. There is a saying that I think is dumb, but applies here. “you don’t know what you don’t know.” I didnt know, I didn’t know so much about transplant lists. What an exhausting trip for all of you. And that is just one of many over the past 19 years. You people amaze me! Thank you for sharing and thank you for the reality check on my own “exhausting” days I have periodically. Love you.

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