When Marian turned one I did not know if she would live to be two. That sounds like an exaggeration, it’s not. I only shared this thought with my very best friends because it was so horrible. At the time we knew Marian probably had a storage disorder, but had no idea which one. Only that lysosomal storage disorders are 100% fatal and 95% not treatable. She was barely starting to crawl, barely putting weight on her legs, choking on her food and was underweight and not growing much. But the possibility that she actually had a fatal disease was too horrible to bear. Everyone wanted to hope that it would be something else, a false alarm, including us.
When she got sick around her first birthday with croup it stayed for three weeks. She coughed one day for 14 hours, every few seconds until it left her limp, sweaty and shaking in her crib physically exhausted from the effort, unable to even lift her head. We brought her back to the ER in the middle of the night, and as we drove I thought, are we ever going to be bringing her home again? Is this the end?
There are so many stories. Twin girls who were a bit underweight, not growing much, just starting to crawl at their first birthday, trouble eating solid foods. They went into the hospital with pneumonia and then they got rapid fire hit with decline and more symptoms. They died months later. It was NPC. I read that story and looked at their faces, they looked and sounded like Marian. I emailed their mom, this is my daughter, her name is Marian, this is what’s happening, do you think it could be NPC? I didn’t hear back. I then felt like a crazy person and didn’t email anyone else. What are the odds it’s the same thing.
Just before her first birthday we had a little mini celebration for Marian at the park with our mommy and me friends. Then she got sick. Then she had her ultrasound finding an enlarged spleen and the world shifted permanently. She turned one and it came and it went, and we were so sad and frightened.
Tomorrow she is turning two. I had thought, if she makes it to two we’re going to have a huge party to celebrate Marian, how well she is doing, how grateful we are. But I can’t do it, I am so happy and grateful but I am still sad and frightened. I am tired. I am scared.
Marian today is a miracle. She has a future. She has hope. She may grow up. Maybe. I believe in my heart that she will. And for now, that is enough to keep fighting, keep pushing for this girl who loves life, is holding to it tight. Who is walking, talking, loving and loved. Toddling over getting balls out of the closet, saying “hi dada,” and “ni night” and pointing to dogs and saying “woof.” Leaning in for kisses saying, “MWAH” and following her big sister around with stars in her eyes.
Who is put to sleep every two weeks and has a huge needle inserted into her spine to keep her alive. Who leans her face into the gas mask because she knows what it is. Whose belly is getting bigger from the cholesterol building in her spleen and liver. Who has to go to the ER when she gets a high fever because it could be viral meningitis. Who is a younger and earlier version of children who are fighting and dying, even on the treatment. Because it’s a treatment, it is a miraculous lifeline but it will run out, and the clock is ticking.
So for today, the day before her second birthday. I truly am filled with gratitude, hope, love and awe for where we are today. I never could have imagined. But, it is still sad, it is still scary, and it is still a long fight uphill carrying a heavy load with tired arms. So thank you. Thank you for carrying Marian forward in little ways as we catch our breaths. We need so much, she needs so much to get there but it is possible. There is urgency, and there is hope.
When we did a news interview before starting treatment I said, “I truly believe Marian will be one of the last to die or the first to survive.” This morphed into “I hope she’ll be the last to die or first to survive.” I don’t hope she’ll be one of the last to die. It's possible, but I don’t hope for that. I hope she’ll live. I see her surviving. I see her living. And turning two is just the beginning.