By Anne Fricke
Freya has Prader-Willi syndrome, which brings various challenges into her life. One of the main challenges is energy level. She can often nap at a moment's notice, and we will find her curled up on her bed or the end of the couch or passed out in the back seat on the ride home from school. \
Along with energy level is the critical need to be supervised around food. For those unfamiliar with the syndrome, people with PWS deal with hyperphagia, an incessant feeling of hunger. PWS is a spectrum disorder, so this affects people to varying degrees. We are grateful that this affects Freya at a very manageable level so far. She has a very good understanding of what her body can tolerate food-wise (pizza and pasta typically cause her belly to hurt, and she will often choose alternatives if that is what is offered) and that people have different needs regarding portion size and how often they eat. It's not always easy for her, and we certainly have moments when food is the central obsession of the moment (and no more parties with charcuterie boards in every room!), but it is always an awareness I must have to keep her safe.
People with PWS typically have low muscle tone. Hypotonia and failure to thrive were her first diagnoses as a baby. She was too weak to even nurse. But now, this girl plays the stand-up bass and carries it to and from the music room and our car. She played basketball last year and made several baskets (only once during a game, but there was quite a cheerful uproar!) She might be a bit slower than most of her "typical" (what's typical, really?) peers and take longer to get to her destination, but she keeps up pretty damn well.
My biggest concern for this trip is the emotional dysregulation that comes from exhaustion, uncertainty, a change in place and schedule, and struggles with social interactions. Other than worrying about how Freya's body will handle hiking at such a high elevation, my main concern for her (and for me, because let's face it, the more dysregulated she gets, the more regulated I have to be, and I'll probably be exhausted as well) is how she will handle this trip emotionally.
I've been thinking and prepping for a few weeks now and wanted to share with you all some of the things I am doing to prepare for this great adventure.
1. Request to not drive other kids in my car.
This was a huge one for me. I am typically a driver on these trips. Like I said, I'm from the Midwest, so not being in a helpful position, telling someone no, is difficult for me. But my thoughts kept returning to a three-day camping trip her class went on at the end of the year last year and how emotionally exhausted I was by the end. (In Freya's defense, she was having a lot of big feelings around the end of the school year and saying goodbye to her teacher, which was compounded by lack of sleep and crappy social dynamics, which have hopefully been resolved.) Driving my own car allows us to go home if things get too intense or difficult. Freya has assured me she will be staying the whole time, and I am certainly open to that. But I am much more relaxed about this trip, knowing that we can go home if things get too hard for Freya.
2. Inform the new parents and remind the familiar ones that Freya needs supervision around food.
Of course, this may be more challenging when there are 20-plus kids together in a group for camping. They have rules about meal times and things, and I will supervise Freya around those times. Still, it is important to let the other adults know that they should let me know if they see a cute little blondie snagging a bunch of granola bars. Thankfully, it's not a situation that has occurred yet, but with PWS, we must always be vigilant in case.
3. Gratefully accept growth hormone from a friend who has a small backlog.
I'm sure this is a controversial one in many ways, but for parents whose kids are taking beneficial medication and have faced the scenario of that medicine not being available, I'm sure you can understand. Families have been struggling to get growth hormone for months. It finally hit us right at the time for this demanding adventure. Thankfully, I have a friend who could supply us with extra to at least get us through this trip. If necessary, I'll deal with the withdrawal afterward at home.
4. Email the Pediatric Endocrinologist asking for energy supplements and doses…then buy them.
Got the dose for carnitine and bought some fancy (and overpriced) electrolyte drink mixes to add to Freya's water to ensure she drinks it.
5. Talk with Freya repeatedly about what this trip will look like.
And remind her that it's okay to step back and play card games at the campsite rather than push too hard to get to the top. Of course, I am leaving that possibility open, allowing that she is a very determined person and often performs beyond any expectations I may have.
6. Try to prepare myself to not have expectations on how this will play out.
I'm conflicted. Part of me is terrified to try and make it to the top, and another is determined to do just that. But this isn't my trip. These aren't my calls (unless, of course, I see Freya heading into dangerous territory.) So, I am trying not to get attached to an opinion either way. Other kids are also going, and I may need to stay back with some of them. It's all on the table, and I am reminding myself of that.
7. Practice my calming breathing exercises and review tips on how to stay emotionally regulated under stress.
Here's the big one for me. Preparing myself to be the support that Freya needs for 5 straight days without much of a break. Thankfully, other parents going know Freya and how to support her, so I'm not totally adrift. But I know some moments will trigger me. Moments when Freya is exhausted and stuck in a perseveration or a feeling and the only way to get her out of it is through. Those moments demand a calm, untriggered, and empathetic response. My skills are getting better, but this trip will be a test.
I hope to return from this trip with many wonderful stories and pictures of Freya challenging herself and connecting with her peers. Whether that is at the top of a volcano or somewhere along the path at a lower elevation, as long as there are smiles involved, I will consider it a success.
About the Author
Anne is the founder of BREATHE, a poet, and mother to three daughters, one of whom has Prader-Willi Syndrome. Much of what she knows about herself, she has learned through writing. You can check out her "Journal Therapy for Parents and Caregivers" course on this website to learn more about it, or watch for any of her live workshops.