“God is our refuge and strength, an ever-present help in times of trouble. Therefore we will not fear, though the earth give way and the mountains fall into the heart of the sea, though its waters roar and foam and the mountains quake with their surging…He says, ‘Be still and know that I am God. I will be exalted among the nations, I will be exalted in the earth.’ The Lord Almighty is with us; the God of Jabob is our fortress.” – Psalm 46
Tomorrow is our fourth Prader-Willi Clinic…a series of appointments that takes most of the day and all of my energy. It is an extraordinary blessing to have a clinic devoted to such a rare disorder less than five miles from my door, and I am thankful for the multitude of specialists who make it possible, but I would be lying if I said I was excited to sit in a room and wait through eight back-to-back appointments with a two-year-old. I get tired just thinking about it. And yet as I write this on the eve of clinic, what I feel most is fear.
In Eliza’s first year of life, the hits seemed to come one after another, culminating with the unexpected news that she had hip dysplasia and needed two surgeries and severe casting. But in the time since that last cast came off (just before her first birthday), we’ve been in a pretty good groove. Sure we’ve walked through some minor setbacks, but I feel like overall, we hit a sweet spot with her weight, her motor planning, even her speech. We have had no hospital stays in over a year, and while last summer’s orthopedic appointment wasn’t quite as good as we had hoped, it was enough to stop the talk of further surgeries for months. But months have passed, and tomorrow will come with answers I don’t know if I’m ready to hear.
I am scared that our sweet spot is ending. And while I’m definitely afraid that the x-rays will reveal a regression in her hip dysplasia and scoliosis that I cannot see, what terrifies me the most is what I can. I see my baby girl transitioning towards the next stage of ceaseless hunger, and I want desperately to reverse it or at least stop it from progressing.
Hyperphagia (excessive huger) is the hallmark symptom of PWS and one that usually creeps in between ages 3 and 8. Eliza is 2 but is exhibiting signs that we are heading to this next phase early. For many months, she has had an increased interest in food, but recently that has spiraled into new behaviors: asking to eat the moment she wakes up and sometimes less than an hour after finishing a meal, throwing tantrums when she can’t eat when she wants, sneaking food from the cabinet if it’s ever left open, and even pulling food out of the trash. I cannot begin to describe the heartbreak I felt when I first noticed her crouching on the floor under our kitchen table to eat the crumbs that had fallen.
This disease is so unfair and so multifaceted that the thought of receiving even a morsel of bad news tomorrow at clinic makes me feel panicked and shaky. On the crest of so much uncertainty in our world, I want desperately to protect my girls…to shield them from all that is scary and sad and unjust. But not only is that not possible, it’s not productive. Hiding my head in the sand and wishing for another outcome will not change the reality awaiting me on the surface, but it might keep me from taking the steps that will help.
So instead, I am praying though Psalm 46, the words of which hang in our stairwell surrounded by family pictures. I just passed it on my way to the nursery where I was headed with a sleepy but happy Eliza in my arms. She was squeezing my neck in one of her famous hugs, reminding me that while she may have struggles, she is overwhelmingly defined by her joy, her love, and her persistence. I was reminded of something else too…that while God does not promise an easy path (I mean, the earth gives way and the mountains fall into the sea in verse 2), He does promise to be with us. I don’t need to know what will happen tomorrow or next month or 50 years from now. But I can rest in the faith that even if it turns out that our journey is leaving the sweet spot and heading for some rocky terrain, the creator of the universe is with me…is with Eliza, whispering gently in the storm (or the calm before it): Be still.