Last night a fellow heart mom posted a link to a study made by the Single Ventricle Survivorship program at Children’s Hospital of Philadelphia. The title included the word ‘relentless’ and was about the Fontan circulation which is what Izzie has. Since March 5, 2012 I have educated myself on her heart defect and read research in hopes that new breakthroughs are on the horizon. When I read these article, I read them with great hope. If hope were balloons, all of them were popped last night.
The article begins with this statement: The Fontan circulation impacts organ systems in an indolent and relentless manner, with progressive decline in functionality likely.
Ok, I knew that her circulation was less than ideal. This shouldn’t be a surprise to anyone. What was a surprise was reading that multiple organs are affected by this, not just her liver. Every Fontan patient will develop fibrosis in their livers- there is no getting around that. It is a natural byproduct of the pressures that have developed from the circulation. Her blood flow to the lungs is passive and dependent on various pressures to get where it needs to go. This high pressure affects the liver, lymphatic system, and inevitably, her heart function.
Speaking of heart function…
Another lovely side effect is a decline in heart function. Well, we knew that. We knew that her right ventricle is not as strong as the left and will eventually give out. What I didn’t know is that it may also have lost the ability to gain endurance. In other words, if she runs, she isn’t building up muscle strength in her heart. We learned from her last hospitalization that something has affected her sinus nodes so her heart rate has dropped in her sleep. These same sinus nodes fire off electricity necessary to increase VO max in the heart that allows for stamina to build.
I was heartbroken to read these things. I want more than anything for her to be able to do the things she wants to do. This doesn’t mean we are saying no to soccer or anything else besides football. This means that she will always lag behind and struggle amongst her peers. There may never be a point where she can be at their level even for a minute. That is what upsets me. I see her face as she watches her sister run ahead of her. I want to pick her up and run with her, but she says no very emphatically when I ask and keeps trying. Sometimes it ends with her coughing to the point of vomiting. Would you like to worry about whether your kid is going to puke in public or not just because she wants to run like her sister? I didn’t think so.
The last message I received from the article is that Izzie will need a transplant. There is no maybe. The only question for her is when. Here is where it gets painful. I try so hard to be open to other families fighting diseases like cancer and the publicity they get with positive attitude. Honestly, it is extremely hard when you feel like no one is understanding that your child’s condition, like cancer, is fatal. She doesn’t have the option to have chemo and then be declared CHD-free. She will always have to worry about herself in a way that most kids don’t. Every illness that affects children is tragic. Izzie’s is tragic too, and just because she is running around right now does not mean that she will be fine ever after. Looks are deceiving. We have learned this painful lesson with her over and over again.
I am not saying that cancer isn’t a horrible disease. DIPG and all of the quick moving tumors deserve to get attention. What I am saying is that heart defects and the side effects of them are also just as horrible. Here are a few in case you didn’t know: poor eating, failure to thrive, neurological issues, constant monitoring, multiple surgeries, having to be cautious when others are sick, needing oxygen, heart failure, issues with lymphatic system, arrhythmia, living life out of a hospital room. This is our life and the life of many friends’ of mine, and when I read an article like this it reminds me of how much more needs to happen for our daughter to have the quality of life we hope for her.
I asked Chris to print this out and I will enter our clinic visit armed with questions about how can we be proactive with these side effects. What is BCH doing to address these issues? Why isn’t there a single ventricle survivorship program like what is at CHOP at BCH? Look out, cardiology….I’m coming and I want answers.