Teetering on the Adolescent Balance Beam With Rare Disease

When entering parenthood, there’s almost always this one, sudden moment of intense fear that comes over all of us. You question whether you’re prepared, if you’ll be good enough, how you can help your child mentally and emotionally navigate the world you’re bringing them into…the list goes on and on. In my case, I blinked my eyes and suddenly my son, Landon, was on the cusp of celebrating his 13th birthday. He’s gaining more independence and it’s a bittersweet realization. As Landon’s medical caregiver, his more recent free-spirited thinking and maturity has caused me to question how to address this next chapter of his pain journey.

• What is Ehlers-Danlos Syndrome?
• What Are Common Ehlers-Danlos Syndrome Symptoms?

• The Mighty’s Caregiving Toolkit

My son began exhibiting signs of fatigue and lack of muscle stamina before entering first grade. When out shopping or exploring, he would find the need to suddenly sit or lay down on the ground. It was the fatigue and discomfort, coupled with his hypermobile joints, that determined that these symptoms stemmed from hypermobile Ehlers-Danlos syndrome (hEDS); each one has taken us on a medical mystery tour. He’s seen physical therapists, a geneticist, motility specialist, cardiologist, nutritionist, pulmonologist, allergist and frequent visits to his primary physician. This is a common list of medical professionals often seen by the pediatric EDS population, though the severity and frequency of symptoms vary from child to child.

If you’re unfamiliar with EDS, it’s a genetic connective tissue disorder, with 13 identified types that impacts each individual differently. Those diagnosed with EDS are born with faulty collagen, the glue that keeps our joints in place and provides structural support for our muscles, bones and organs. In my son’s case, the symptoms have manifested primarily in the form of headaches, stomachaches, decreased energy, dizziness and, more recently, stability issues and pain in his neck and legs.

A walk in the woods or an afternoon playing outside almost always leads to Landon spending the rest of the evening using ice packs, heating pads, requesting gentle massages and laying down. Historically, our medically complex family coordinates these post-crash periods by declaring a “lay low day,” 24 hours of hydrating, resting, icing and heating troubled areas of the body. Now that he’s almost a teenager, Landon has expressed being OK with the EDS flare-up repercussions and foregoing a day of rest so as to not miss out on activities with peers or family.

I admire his bravery, as most adults wouldn’t consider sacrificing comfort for enjoyment and eventual pain. This heartfelt, verbal applause I’ve recently shared with my son about his courage has shifted, however, from the proud parent to the practical caregiver; sometimes I really dislike playing that role. This position has its own set of unique rules and challenges outside of the typical parenting responsibilities. Along with the decision-making guardians make for the wellbeing of their child, the pediatric caregiver is deeply connected to their child’s physical health and hindrances. This innate mindfulness is a double-edge sword. It’s powerful enough to cut through the act your child puts on for the outside world in a desperate attempt to fit into society’s definition of normal. The double-edge sword enables caregiving parents the ability to calculate potential risk outcomes; we’re constantly running “what-if” scenarios with and without setting some sort of safe limitations. The heavyweight responsibility rests on the caregiver’s shoulders to help their child understand and identify coping mechanisms to accept the reality that their body is beautifully diverse from the community at-large.

So as not to draw much attention to his EDS diagnosis that gives the condition too much power over his life or make him feel in some way deficient, our family has worked together to find adaptable solutions; Landon is also becoming a brilliant problem solver on his own. This newfound ability gives him the confidence to engage in planning out his homeschool days, take breaks on his own, grab ice packs, or use a cane. He’s still working on his communication skills when it comes to letting us know when his pain is starting to escalate, but I believe he’s trying to figure out if and when he can care for himself.

Lately I’m at a loss with my caregiving role because Landon is entering adolescence. In the years leading up to now, I’ve been able to channel into my caregiving responsibility, despite the extremely heavy heart it has come with, in order to assess, evaluate, and respond. Why is it suddenly so difficult for me to use the word “no”? This unfamiliar feeling had me questioning my parenting/caregiving techniques. Should I be giving my son sole responsibility in choosing whether he wants to engage in physical activities presented to him, knowing full well he’ll crash later in the day? Do I continue welcoming and hearing his thoughts, but then make the ultimate decision on his behalf? Is there a fair and compromising option our family hasn’t thought of yet?

If a fellow pediatric caregiver were similarly stuck in this parental/caregiving purgatory and asked for my input, I’d tell them to consider approaching each situation as its own individualized opportunity to maneuver. Just as their child is distinct from others, such is the case for each physical activity that will present itself over the years; their child’s current health status and energy levels are also ever-changing. I’d advise that they can plan to approach each circumstance semi-consistent with one another, but to be flexible – a word familiar to all EDS families. This approach, however, should then be shared with the pediatric “zebra” (an endearing term to honor a person living with a rare disease, especially those with EDS) so that the child isn’t confused as to why one physical activity may be OK one day, but not another. For the most part, this flexible process has been working for us, that is, until the more recent and inevitable adolescent resistance surfaced.

Through no fault of his own, Landon is in the midst of finding and building upon his identity, conceptualizing and connecting dots to better interpret his surroundings. Thanks to the brain’s natural development and growth around this time period, my parenting and caregiving roles are being more closely observed by my pre-teen. This latest type of response is not to be defiant, but is helping him to understand appropriate social interactions and reasons behind decision-making. I’m presently trying to be a supportive spotter for Landon as he teeters on this figurative balance beam he finds himself on at the moment. I’m also attempting to take a safe distance back so I’m there if he falls while also letting him know I encourage his quest for independence, love his unique capabilities and value his individuality.

I know Landon is doing his best at this stage in his life to find that harmony between his maturing and having an unpredictable pain condition. And while I’m attempting to figure out what my role is as he ages, this phase isn’t really about me; it’s about my not-so-little zebra. I’m hopeful that I can pair my knowledge of his health condition with his natural gravitation towards independence and problem solving, using both of our strengths to proactively shift towards becoming the next type of caregiver he needs and deserves.