The Most Common Rare Syndrome You've Never Heard Of

Saturday, May 31, 2014

To Compare is to Devalue--Heather Cole and Harmony Harkema (SILs)

Heather and Harmony and Lili

Harmony's Voice:

The Perfect Family

“I’m pregnant.”
The words came without warning. She simply dropped them into a lull in the conversation as though she were making an everyday observation. She might have said it was supposed to rain the next morning. Her tone was light and perfectly calm, her expression serious.
I was stirring something in a pot on the stove, and I turned abruptly, my wooden spoon leaving a trail of liquid across the kitchen tile. I looked at my sister-in-law intently, searching for a hint of humor beneath the surface, a sign she was messing with us.
There was none.
“You’re . . . joking?” I asked, falteringly.
She shook her head.
Heather’s pregnancy came as a shock. She had thought her family was complete. But God – he was on another page. He had something special in mind for Heather, Jon, and their two girls. Or rather, he had them in mind for someone else.
Seven months later, after months of morning sickness, a challenging bout with gestational diabetes, and burgeoning worries when third trimester ultrasounds showed that Heather’s placenta was calcifying, Rhyse Addyson Cole entered the world. He came quickly, both during labor and at the end of it, his father catching him like a football as he exited the birth canal in a rush of life-giving fluid.
We went to the hospital the afternoon of his birth, the entire extended family crowding into Heather’s postpartum room, passing the newest member around like a shared treat. He was the tiniest baby I’d ever held, a mere five pounds four ounces, with swirls of surprising red hair that led Heather to joke that maybe he was really the mailman’s son. Jon smile was the widest we’d ever seen it.
Rhyse charmed us all that first afternoon, his tiny, perfect face relaxed and calm, his eyes seeming to take the measure of each of us like a wise old soul. Both Eli and I snuggled him close and smiled at Heather’s hints to us that Rhyse needed a cousin close to his age. We had no idea that we were already pregnant. We drove home from the hospital feeling nothing more than a deep thankfulness that this longed-for and deeply loved little boy had safely arrived.
My phone rang just before 5 a.m. the next morning, pulling us from deep slumber. When I saw Jon’s name on the caller ID, I came fully awake with a jolt. Even before I answered, I knew there could be only one reason for Jon to call so early. Something had to be wrong with Heather or the baby.

The days and weeks that followed were filled with mystery as the doctors searched for an explanation for Rhyse’s unique medical state. A fearsome low blood platelet count spurred the hospital OB to rush Rhyse via ambulance to the nearest children’s hospital. Finally, after six weeks in the NICU that involved countless tests, numerous platelet transfusions, and all kinds of other medical intercessions no infant should have to endure, Heather and Jon received a tentative diagnosis, one that would require further testing before it could be made formal. Rhyse had Noonan Syndrome.

Noonan Syndrome. None of us had ever heard of it. Heather and Jon had to educate themselves out of necessity, and they did their best to educate the rest of us in turn. In the end, all we could really be sure of was that nothing was certain. Noonans looks different on every child, and we had no idea how it was going to look on Rhyse.
It’s been over two years since Rhyse was diagnosed with Noonans, and it’s safe to say he’s become the light of our collective family life. He has proven himself hardier than any toughened adult, undergoing constant assessment and treatment for everything from feeding issues to breathing problems. He has a team of twelve specialists who are regularly reassessing his needs. He has a G-tube. He gets twice daily steroid shots to help him grow and will continue to do so until he’s 18 years old. He gets nightly breathing treatments and will soon have both his tonsils and adenoids removed in order to improve his ability to breathe while he’s lying down. But these medical pieces, while they are a big part of Rhyse’s life, do not define him. Far from it.

What does define him? The same things that define any two-year-old. Bottomless curiosity. The desire to make his own place of significance within his family and his world. That unmistakable toddler mix of courage and timidity. The hunger to be heard, understood, respected, and valued.

When we think of Rhyse, we don’t think of Noonan Syndrome. We think of our spunky red-headed nephew with the unshakable sense of humor. The one who loves and fights with his older sisters like any little brother. The one who loves to tease his daddy. The one who, in spite of delayed verbal development, communicates his needs, wants, and feelings as clearly as can be. The one who loves cars and trucks as though it’s in his DNA. The one who is bright and intuitive and can read the atmosphere in a room as well as any psychologist. The one who wants, just like any other child, to be loved and snuggled and teased and talked to.

Rhyse and Lili
We live halfway across the country now, and most of our time together consists of sending each other videos or making time to Skype. When we do, we don’t see a sick child, or a struggling child, or an abnormal child. We see a happy, active little boy yelling and running and playing and just having a ball being alive. He and our daughter, Lili, who is nine months younger, wave to each other gleefully across the miles, always excited to see each other, even if it’s only via a computer screen. Indeed, Lili’s favorite “TV show” is Rhyse’s YouTube channel.

And his parents? His sisters? No one, no one, could love Rhyse, could care for him, the way they do. Even at the end of a week wherein there have been gas bubbles in the G-tube and episodes of throwing up formula all over the minivan, hours spent in a specialist’s office that end in yet another tweak to the care plan, and midnight visits to the emergency room - even when it feels like there’s nothing more that could possibly go wrong, none of it can cancel out the love shared between the five of them.

Noonans doesn’t stop them from being a family who goes to the library and the bookstore and the zoo and the beach. A family with a swimming pool and a trampoline in the backyard. A family who goes on hikes and bikes rides and trips to the ice cream shop down the street.

It’s still not clear what Noonans will mean for Rhyse long term, what he’ll be able to do or be as an adult. What is clear, however, is that Rhyse is exactly where God meant for him to be – cradled in the bosom of a family that, while as imperfect as every other family, is perfect for him.

Harmony Harkema is the proud aunt of Rhyse Addyson Cole, a spunky little boy who just happens to have Noonan Syndrome. She is also the proud mother of Rhyse’s favorite cousin, Lili. Harmony lives in the Washington, DC area. She blogs at www.beyondtoast.com.


Mom's (Heather) Voice:

Mom and Rhyse(PTNP11)
When I walked into Meijer, a mid-western mass retailer, I found myself settling my eyes on a little boy who has a syndrome.  He and his dad were walking along the fresh vegetable aisle holding hands, and sporting smiles spread from ear to ear.  I didn't have my son with me at the time, but as I sauntered by these words came to my mind, “I know.”   I continued on past the salty snack aisle, the baking aisle, and the ethnic food aisle and onto the cleaning aisle to grab some toweling. But all the while I desperately wanted to run back to that daddy and tell him, “I value your son too.” I grabbed some toweling and made my way back to the front of the store, purposefully gazing down each aisle momentarily to look for the boy and his dad.  I never found them.  I feel I missed an opportunity to remind that dad he’s not alone.

According to the Merriam-Webster dictionary the second meaning of compare means “to look at (two or more things) closely in order to see what is similar or different about them or in order to decide which one is better.” The second definition of devalue means “to cause (something or someone) to seem or to be less valuable or important.”

All of our lives we compare who we are to who someone else is or what we may or may not have as compared to a friend or a stranger —wondering how being like someone else or having what they have might define us differently.  These comparisons seem inherent to our nature as humans, though for each of us these may be bent in different ways according to the culture and sub-culture that we live in, as well as our personal spiritual journeys.

Comparisons can push us to greater success, or greater failure. Comparisons can be inane, or horrifically self-defeating. If we could live a life without comparing I truly don’t know if it would be better or worse! 

In the beginning of our journey’s with Noonan Syndrome I believe many of us could only see the blatant differences in our children.  And those differences were painful.  Most mommies were being wheeled out the door of their birthing center with their little bundle in arms, overwhelmed with the impending sleep deprivation and life changes that come with having children.  But at least that is “normal.”  For many of us NICU instantly became our “normal”. Instead of exuding joyous emotion over our new arrival, in between yawns of course, we had to find a new way to handle both joy and pain in the same moments. We had to learn quickly to allow strange men and women to handle, cuddle, feed and clothe our newborns.  And trust doctors we had never met.  We had to learn to fore go the expectations of a new parent, and learn to return home from the hospital each day empty handed.  It has been two and a half years since my journey with Noonans began and the wounds that these things left can easily be torn open from sheer memories alone. I can only look back for a short time, and then I must move on to savor the present—to value my son for whom he is.  If I don’t value him, as a parent, for both his differences and similarities to others I don’t know that he will be able to value himself as he becomes more self-aware.

When others compare our child/children to the mainstream or to other non-typical children we feel devalued as parents, and it feels like our child/children are being devalued as well.  But if I could gather all of us parents together in one room I think we could agree that as our love for our child grows deeper, we focus less and less on their differences. And whether others compare our children to the mainstream or we ourselves compare our own children to the mainstream it is fruitless, and it can devalue everyone.  As moms and dads of children who do not fit into the “typical” category, whether the differences are physically obvious or not, we know that comparison is less about differences and more about commitment: commitment to love beyond the imperfections of humanity, and to love and to value in a new and deeper way.

Maggey, Rhyse, Leah
I wish I could say I was perfect at commitment instead of comparison myself, or that the words of others comparing my children just slip off me like water down a slide.  But I can’t.  As of mother of an adopted daughter whose inherited skin color is a deep, lustrous brown I face the challenge of rising emotions when she is compared to white folk. Just this past week she came racing off the bus upset that she was called, “dark chocolate” all the way  home. My second daughter is white and “typical” in every way and truly can’t understand why others tease her sister—they are the best of friends. My son who not only has Noonan Syndrome but has spectacular, orangey-red hair--for which he gets gawked at by older ladies, just plain sticks out wherever he goes!   

While we do not want to be hypersensitive to the stares or the questions we get from strangers I will be the first to raise my hand and admit I get tired of the “he’s so small what’s wrong with him?” question or “your son looks tired is it nap time?” And don't complain to me that your child won't eat his veggies!  At least he eats!  Ok, see?  I, too, have to take a big breath and bite my tongue on occasion.  Rarely does anyone really want to know about a syndrome, and when I use the word, “ptosis” their own eyes glaze over!  There are days where I am tired of feeding Rhyse through a g-tube, being puked on at the most inconvenient times, changing bed covers three times a night from med port eruptions, perpetual sleep deprivation and a long list of diagnoses that only continues to get longer-- I struggle with feelings of incompetence and uselessness as a person.  And in the wee, wee hours of the morning, when I can’t relax long enough to ooze back to sleep because the sounds of Rhyse’s asthma are keeping my nerves sharp and edgy, I desperately wish Noonans away like the whole thing was just a hokey nightmare from too much pizza. I crave “typical.” Eventually morning does come, and the brain fog lifts, and there it is, Noonans, still staring back at me in face of a beautiful little boy that I could not love any less, no matter what. 

So I erroneously compare myself with that mother "out there" who has healthy kids, who keeps a perfectly clean house, and always gets the dry laundry out of the dryer before wrinkles set in--and I come up so far from human.  Being a mom of a child with a syndrome  makes me feel different too. I guess we all have to keep reminding ourselves not to compare! We compare and we make assumptions; assumptions that are usually wrong.  And for some darn reason when we compare and assume we aren't thinking in the best interest of others! 

I feel I must interject an important point here: this is from a mother’s perspective.  I am not the one who is labeled “different.”  I am not the one who has to overcome physical and emotional challenges in a world set up for “typical” people.  I grieve EVERY time I read the many accounts of kids, written by parents of kids with Noonans, being viciously bullied because of their small stature.  I am moved by the love of a sister when I watch Alexia Grace Hopkins singing, "DifferentThan Everyone Else", a song she wrote in honor of her sister who has Noonans.  In a bittersweet way I enjoy reading and rereading Lee Robinson’s blog regarding his beloved brother, Connor.  The paradox between resenting Connor because of how his medical issues inherently brought so much attention as a child, and yet the fierce love of a little brother for his big brother gets me every time. I see how this could be playing out in my own kids’ lives. And yet there is nothing I can do to change those dynamics.  I can only love equally and believe someday all my kids will understand.

Each time kids who have NS speak out I listen and learn.

So as I take a deep breath and examine my own coined phrase, “to compare is to devalue”, as a mom who has a child with a syndrome, I challenge myself to always value him well beyond any reason to compare him to others, and to teach my other two kids that comparing themselves to others for any reason is futile.  And most of all to help Rhyse "catch" the understanding that being different doesn't make him less.  Not to mention kicking myself in the pants occasionally and reminding myself of the same thing!

Heather and Jon have three kids, Leah, Maggey and Rhyse(PTNPII). They reside in Allendale, Michigan. Jon works as a Materials Manager for an international company, and Heather is a stay at home mom--who occasionally likes to blog!   Life Journey's



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