Saving Celise

The ICU rollercoaster ride continues for Celise, as she experiences both progress and setbacks at the same time. It’s strange to be both excited and concerned for her. Her heart continues to improve, and it is significant enough for her doctors to be cautiously excited—and to be honest, surprised at how quickly they’re seeing results! Meanwhile her respiratory infection has flared up yet again, causing her to go back on high support on the ventilator and a few days of paralytic. 

Everyone has been celebrating, because as of yesterday Celise’s BNP is finally within normal range! We knew BNP would be the first place we’d see the effects of her trial drug, but we couldn’t have imagined it would work so quickly. With a starting number of 6,578, just five weeks of the trial drug and it is now 84. Her doctors are thrilled with these results.

However it’s hard to feel celebratory when we’re also so concerned about her respiratory infection. She has been infected with this bug since just after she was born, and it’s the main reason she has needed the support of the ventilator. Unfortunately it is an antibiotic resistant strain that does an especially good job of sticking to plastics, like her breathing tube, so it has been impossible to completely annihilate. Every few weeks it flares up and we have to put her on another cocktail of drugs to kill as much of the bacteria as possible. This flare up is particularly disappointing, as it comes right as Celise was starting to get the hang of her ventilation trials. Her doctors have a plan to switch out her breathing tube at the end of this course of antibiotics, in hopes that some fresh plastic will buy her enough time to be extubated before the bacteria has a chance to grow back again. 

Celise has had two new echocardiograms since the last entry, and the progress is quite remarkable. Her doctors weren’t expecting any noticeable changes on the echo until she had at least 2-3 months of the trial drug in her system, but by 3.5 weeks they were already noticing some slight improvements. Now at 5 weeks they can already say that her pulmonary stenosis is no longer a concern! When we arrived, this particular issue was considered moderate to severe, and now they’re calling it mild. Her most critical issue, hypertrophic cardiomyopathy, is now being called moderate to severe, and when we first arrived it was extremely severe. Her heart is actually shrinking just like we want it to, and in record time!

All in all, we have a lot to be excited and thankful for, despite the fact that Celise feels absolutely miserable right now. We have to hope that the new tube will keep the infection at bay long enough for her to be extubated. Once she’s off the ventilator, I think she’s really going to show us just how sassy she is. And of course, we have to hope that her miracle drug continues to work so well. It is terrifying to think of where we’d be without her doctors and nurses, and the amazing folks who discovered this alternate use drug. We are so grateful for the support of the MUSC staff and all of our family and friends who continue to check in on us. Thank you, forever.