“We just want a better look…”
Benton was the sixth child to be diagnosed with Bainbridge-Ropers Syndrome in 2014. Seattle, Washington is the place he calls home, and this is a little bit of his story, as told by Benton’s mother, Tamara.
“We just want a better look,” they said during our first ultrasound in 2005, ordering immediate additional exams. Huh? We saw exactly what we came for—anatomy denoting the gender of this miracle life developing in utero. We’re having a boy! What more did they need to see? They murmured to one another. They looked nervous. Uncomfortable. In an instant, my insides shattered. And so began our journey down a path no one would choose.
They believed our unborn son had Trisomy 18 and wouldn’t survive the pregnancy. How could this be?! My husband and I were pictures of perfect health. An amniocentesis proved the docs wrong. Our son did not have Trisomy 18 or any of the other conditions tested in amnio. Yet every ultrasound produced the same nervous discomfort amongst our medical team. With no proof that anything was truly wrong, Dennis and I held hands and bet on our boy.
In May 2006, Benton was born full term via emergency c-section due to delivery distress. The sideways glances between the OR team signaled that a Mayberry life would not be ours. Craniofacial abnormalities. Contractures. Global hypotonia. Undescended testes. Labored breathing and feeding difficulties. Several things “wrong” that collectively didn’t form a clear picture. They had a medical mystery on their hands. Upon discharge seven days later, the only thing we were told with certainty was that we were going to need to advocate for our son.
This was a very dark time for us. Why was no one advocating for us? You just sent first-time parents home with a newborn with severe disabilities. You gave us no manual, no map, no confidence. We’d never raised a baby—we didn’t know what we didn’t know. And what we didn’t know then was that Benton was in way worse shape than the delivery docs ever imagined.
We quickly earned our frequent flyer status at Seattle Children’s Hospital, visiting regularly with nearly every clinic, but claimed by none. Diagnosed with ‘failure to thrive’, Benton received his first feeding tube at six weeks. He finally started to gain weight but was lethargic and unresponsive nearly all the time. With a “floppy airway,” he’d been a noisy breather since birth, but it grew worse. Fast. At just over one year old, Benton had an emergency sleep study that was followed by a phone call that would change our lives. Benton needed a tracheostomy. NOW. Granting docs permission to punch a hole in our child’s neck was absolutely terrifying. A trach opened up a whole new world of risk, but for the first time ever, our son could breathe and sleep peacefully.
It was around this time that we stopped asking “WHY?” Why us, why our son, why, why, why. Did I give this to him? Did Dennis? Was it our combined genes? Could we have prevented this? Did this happen during IUI (yep, unexplained infertility, too)? Are we paying for a sin we committed earlier in life? Why, God, why? One day we realized we’d never know the answer to why. And even if we did, it wouldn’t change our reality or responsibilities. Letting go of that question lifted a big weight off us, triggering a mental and emotional shift that invigorated our fight. Our advocacy.
Several years of intense assessments, therapies and appointments followed. Benton was profoundly developmentally delayed and completely dependent on trained individuals for his 24/7 care. Nurses began floating in and out of our home. He missed every typical milestone. When not working full time or taking Benton to appointments, we battled for support from private insurance, Medicaid, the school district, even our medical provider network. All the while, Benton received genetic testing, but nothing produced a positive result. We made peace with the fact that we may never have a diagnosis and let Benton write his own prognosis in real time. At 3, he was able to remain upright when placed in a seated position. At 6, he began taking some blenderized foods by mouth. At 7, he started to bear weight. And at 8, he finally got the word we’d stopped waiting for…
In December 2014, Benton became the sixth person in the world to receive the Bainbridge-Ropers Syndrome (BRS) diagnosis, a mutation of one of his chromosomes 18. He was also the oldest in the bunch, so he instantly became a prognosis poster child. The geneticist could tell us nothing more about BRS except that it was non-degenerative. That’s all we needed to hear. We didn’t need this diagnosis to tell us Benton would never have the life of his typically developing peers, but it was a relief to learn he was expected to live. So we went back to writing his story, day by day, one page at a time.
At the time of writing this post, Benton is 12 going on teenager (oh my!). With nearly 200 individuals now diagnosed with BRS worldwide, he is far from the oldest but is one of the most profoundly affected. He cannot talk or communicate via devices or sign language and has severe strabismus and vision problems. He cannot run or jump, but with the help of orthotics and equipment, he can now stand and walk assisted for short periods. Though his G-tube remains for liquids and meds, he takes nearly all his blenderized food by mouth and is starting to self-feed. He’s pulled through a wide spectrum of major surgeries from dental work to bilateral flat foot reconstruction. Managing a group of nurses in our home is like a second job, but they’ve been instrumental in Benton’s care and development; they are his angels on earth.
Dennis and I work hard to give Benton life experiences he couldn’t have without our help—stuff the rest of us take for granted. We choose to believe there’s a lot going on in that noggin of his and are hoping that someday we’ll discover the magic therapy or tool to unlock it and let it flow. Benton loves interacting with people, laying under the trees, feeling the breeze and cuddling with his little brother Iron (9). He continues to defy expectations and fill us with intense pride.
Make no mistake about it, life with Benton is hard. Really fucking hard. But it’s ours and we’re navigating it the best we can, one day at a time.
Benton has inspired us to see wider, feel deeper and live life with more intention. So when people ask how we do it, we simply look at Benton and offer the only answer possible: How do we not?