Last night, I was standing at the sink in my house getting a glass of water. Suddenly, the smooth feel of the glass in my hand triggered something, and I turned and hurled it through one of our kitchen windows. The glass and the window shattered, spraying shards everywhere. I picked the pieces up bare-handed, deliberately letting the glass cut my palms and fingers. Watching my bright red blood drip onto the floor gave me a sense of calm.
Flash back to me still standing at the counter, holding the glass, eyes glazed over as I imagined the satisfaction of breaking something, hurting myself to penetrate the numb shock in which I was cocooned.
I went 30+ hours with no sleep. I stayed up all night with James, making the call to take him to the hospital at almost 4am Wednesday morning. I stayed by his side through the painful needle jabs, failed PICC line placement, echocardiogram, ECG. I talked to his intensive care doctor, his nurses, his specialists, our social worker. I did it all through a haze of no sleep and intense fatigue. By the end of the day, I finally understood what it meant to sway on your feet from exhaustion.
It was then that the cardiologist came and delivered the news of James’ HCM.
Hypertrophic cardiomyopathy is a thickening of the heart muscle. It leads to a stiff heart that doesn’t relax and fill well. There isn’t really a great treatment for it, so it is generally medically managed by keeping the heart rate low. This is accomplished with beta blockers. If those don’t work, surgical management can help – either open-heart surgery or a heart transplant. Unfortunately, getting a heart transplant is hard when you don’t have trisomy. It’s not even an option for a trisomy baby.
The reason that this is huge, life-altering news for James is because it means that he cannot have heart surgery to repair his heart defects. No surgeon will do open-heart surgery on a trisomy baby with HCM. Another reason that it is huge news is because HCM has not previously been linked to trisomy 18. As a result, the cardiologist is treating this as a finding independent of James’ trisomy. If it is truly unrelated, then it is either a spontaneous mutation or genetic. If it is genetic, then my husband and I must have the gene, and thus, our girls could also be affected.
It was a lot to absorb in a state of utter sleeplessness and also hunger (I didn’t eat all day other than a donut and some coffee brought by my friends). After the cardiologist left, I sat in the dark PICU room, holding my finally sleeping baby, and wept. I cried hot tears onto my sweet son’s face, thinking about losing him, thinking about his sisters at home. I sat there in the dark a long time, lost, alone, and afraid.
When Jim arrived, we cried a little together, and then I came home. I hugged my girls, ate some dinner, and crawled into bed at 8pm.
This morning, I start over.
Because really, what’s different? James was born with a life-limiting diagnosis. He wasn’t supposed to live to be born. Then he wasn’t supposed to live long after he was born. Then he developed heart failure, way earlier than anyone expected. He came home on hospice, predicted to die within weeks. Instead, he gained weight and thrived under the loving attention of his sister and parents.
So far, James has written his own story, trod his own path. He has brought joy to his family. He has touched people on the other side of the world with his bright eyes and open spirit. Nothing has changed. He is still who he is. We are still his doting parents, ready to give him every opportunity for a happy, fulfilling life. His sisters still adore him.
He was never supposed to live in the first place, yet here he is.
His palliative care doctor gently reminds me that James will write his own story, choose his own path. Our responsibility as his parents and his caretakers is to follow his lead, allow him to guide us in what he needs, and to ultimately, respect that we did the best that we could with the information we had and gave James his best life.
None of us know the number of our days here, so in focusing on James’ diagnosis, on the bad news, we forget the good news – the miracle of living – being here on this beautiful planet – every day, all around us. The miracle of being here to experience this life. Any of us could die at any time. In that way, James is no different than you or me or anyone.
To this new development – I say, so what? It doesn’t change anything. We’ll keep on loving our little man and cherishing whatever time we have with him. Despair has no place in our world.