Thursday, Gabriella and I got up early and flew to Ontario, CA for her monthly clinic appointment at Loma Linda. Deepest thanks to an anonymous donor, Gabriella flew for free which helped a ton! Being gone for so long back in March was difficult, not only emotionally but financially, so a quick and cheap turn around trip was perfect for April.
We met with a doctor we had not yet met yet, but he knew all of Gabriella's story. The best thing is how connected their whole transplant team is. Regardless of the size of their program, they all work together so closely that they are all familiar with the kids. This doctor is the ICU doctor who will tend to Gabriella post-transplant, and I add him to my list of amazing doctors at Loma Linda! We spoke in more detail than a normal visit about her situation. Since Restrictive Cardiomyopathy is the rarest form of childhood cardiomyopathy, there is still so much to be learned about it. He said most kids with heart defects wait until things get severe to critical before they list for transplant, which is why you will normally see kids on feeding tubes, oxygen, etc, in the stories of heart kids. They try and get mileage out of the old heart before jumping to transplant. Post-transplant is nothing to take lightly... many people think the problems fixed, but it is just an entirely new set of serious lifetime issues. RCM (and other heart defectes) can cause Pulmonary Hypertension... basically can harm the lungs. Let this get serious enough, and treatment becomes very difficult. With RCM, it is a near definite that this effect on the lungs will be severe and fast, and he is now applying this scenario to Gabriella. Combine that with the high risk for sudden cardiac death associated with Restrictive Cardiomyopathy, the general opinion is "getting mileage" out of her heart is definitely not the smartest option.
What do we do about this? Well, he wants to do another heart cath. Typically, their protocol (as well as most other transplant centers) is to have this cath done every 6 months while in the preparation or waiting stages for transplant. Gabriella had her stroke, which halted that from happening at the 6 months mark. Tomorrow will be one year since her first one, so I knew this was coming. Doing a heart cath will check for any signs of pulmonary issues, which will better help them to treat her at transplant instead of going in to find a surprise issue. My immediate fear is another stroke. We talked about that fear, and he is not taking it lightly. We have to decide what the smartest of 2 evils is. Do a cath to better prepare for transplant but risk another stroke? Or skip the cath and eliminate added stroke risk but walk into surprises at transplant? I am not in opposition to the cath whatsoever, but I need to make sure we are all fully aware of this stroke risk and that doctors have a plan set in motion before we do it on how to prevent or treat any clots that might develop. You know me... I'm "that" mom that will question and investigate everything.
The next part of this hard decision is listing. He thinks Gabriella needs to be bumped to a 1B from level 2 listing, being a bit higher in 'importance' for lack of a better word. I am sure the cath will do a lot of the deciding for them, but this might be a decision they make prior to cath. Since her cardiomyopathy is still a very cloudy diagnosis for the pediatric heart worlds and so much is unknown, he thinks the safer bet is to list her higher. Her heart is unpredictable to even these amazing surgeons who have been playing with hearts for decades. And for the same reasons I said earlier about not waiting for transplant, listing as a level 2 doesn't make things go any quicker for her.
He is taking it before the transplant team this week. He said that he thinks Gabriella's situation needs to be looked at a bit closer and in more detail since this has stretched far beyond our original expectations last summer. They will be getting back to me and hopefully we can make the best choice together for the best interest of Gabriella. And of course, I will share the results as we have them.
And the best part of yesterday was hanging out with our new friends. I was introduced to Shannon and 2 girls before our last trip and we met up back in March. We spent the later part of the day just hanging out which was a super nice way to perk up our dreary appointment. We were having such a nice time we got sidetracked and we almost missed our flight! But I performed a travel miracle and made it there on time, only to find out our flight was delayed.... lol.