Data is Power, Ignorance is Bliss
It was about a year and a half ago that Caleb was about the age that we thought he could tolerate a Cardiac MRI without sedation. He did amazing, partly because of him, but also because of the amazing staff that helped him to not be scared. That was on a Tuesday. On Wednesday we spent all morning at our typical coordinated clinic where he sees all of the specialists. The results of the MRI were not yet available. The appointments were difficult for Caleb as things become tougher for him and we grappled with a fairly invasive foot surgery. But what we didn’t know we found out through My Chart at 5:30 am the next morning while I was laying in bed back in Minnesota.
“There is delayed myocardial enhancement starting at the level of the base and extending into the mid chamber noted in the inferior and infero-lateral segments and extending into the antero-lateral segments. There is greater than 50% myocardial involvement.”
I broke. I didn’t have much idea of what it meant, but I knew that any cardiac issues at 9 years old, is just far too young. And while I didn’t know the significance of the results, the “greater than 50%” part hit me hard. I knew it wasn’t good.
While I appreciate the immediate gratification of My Chart test results, I was once again left with these results and google until I could talk to our cardiologist. I read things like:
Death of the heart cells
Myocardial involvement is one of the main factors for mortality
Heart failure
And I googled things like:
Life expectancy for a 9-year-old with greater than 50% myocardial involvement
Quality of life with heart failure
Then I got smart and stopped the downward spiral and reached out to our Cardiologist. It’s been extremely important to me that our clinical care team is more than just a group of physicians that we see a few times a year. That is why we endure long travel days to Ohio from Minnesota, so that we can continue the relationships that we have built for so many years. So I texted our Cardiologist and within minutes had a call back. More than that, she was on a morning beach stroll with her husband while on vacation. I will always make sure we have the right people on our care team, people I know will have my back, and more importantly, Caleb and Dunky’s back.
This conversation was extremely beneficial, not because she said, “it’s okay” or “he’ll be okay” or “it’s no big deal” but because it was a conversation of her first listening to me cry, but then she said things like, “I’m so sorry”, “it’s a terrible disease” and “we will start more heart meds now”.
So here we sit a little over one year later with more data for both kids. Caleb has since had 2 more MRIs, he’s moved from watching Disney movies while getting his MRI to listening to Eminem. They are both now on 3 different heart medications (lisinopril, eplerenone and carvedilol) and we are increasing doses periodically as they grow. Caleb’s results are stable, hoping that the meds are helping to not have additional cardiac issues, at least for now. Stable is winning in Duchenne.
Dunky has had one MRI and his initial results were not good either. In fact, his Left Ventricular Ejection Fraction (LVEF) was worse than Caleb’s initial MRI. It read things like “low-normal” instead of “normal” and “approaching 50% myocardial thickness”. Still scary words and scary stuff, but data is power, and I was ready for this news. Thank you, Caleb, for paving the way so I could take this news better. I still cried, I still yelled, I still grieved this news, but I was able to recover a bit sooner. These results caused the Cardiologist to put Dunky on all the same heart meds as Caleb. He will have another MRI in about 6 months. We pray for stabilization in the LVEF (current state was 53%).
I’m grateful for technology, for EMR systems that talk to each other and for access to information. Duchenne is an interesting disease. I feel like we live in a community focused on data, especially with so many clinical trials in place for Duchenne. However, at the same time I feel like we live in a place of ignorance because there is still so much we don’t know about Duchenne. Like the Cardiologist told me while strolling that white sand beach, “there can be a kid with Duchenne in heart failure that stays at that level for years” but I can’t help but think about the 12-year-old with Duchenne that died of heart failure.
Yes, 12-years-old. Caleb turns 11 in two days. This is the unknown. People love to talk about adults with Duchenne that lived into their 30’s, but it seems that there are still far too many kiddos with Duchenne not making it to their 20’s. There is so much we truly don’t know. I went so far to ask at our last appointments “how much longer do you think we have?” and that question couldn’t be answered. They don’t know.
So what do we do with this data, with this power, with this ignorance and with this bliss? We embrace it. We use what data we have to make guided decisions on medications and how we spend our time. We use the unknown to treat life as if we may not get tomorrow. We stand back up when we feel so kicked down. We ask for help, we spoil them, we live our lives in a way that we won’t have regret.
Thank you to this village that helps us on this journey.
10/26/24
