Evren Ayik has acid sphingomyelinase deficiency (ASMD), for which he has received expert care from specialists at a children’s hospital in California. Now Evren has reached his twenties, the time has finally come for him to move on to an adult hospital. Evren’s mother, Kara, describes the emotional ups and downs of a fifteen-year-long journey and what it feels like to say goodbye to a place that will always hold a special place in her heart

By Kara A. Ayik

If you take the Avenue 9 exit just off California’s Highway 99 and a few miles north of the city of Fresno, you will drive on a two-lane road past several miles of nut and fruit orchards, grapevines, oleanders and pomegranate trees. And just as you approach the end of Avenue 9, you round a slight bend and suddenly your eyes encounter a magnificent sight straight out of a story book. High on a hill overlooking Fresno sits a giant complex with its central buildings shaped just like a fairy-tale castle, all painted in the soft, pretty colours of ice cream and cotton candy. Driving into the entrance to the complex, huge jungle animal topiaries flank either side of the driveways, and drivers are guided by signage with images of a cheerful blue giraffe named George. It seems to be a magical place.

It is this place that evokes deep and powerful sentiment from deep within me unlike any other place on Earth. What I feel for this fairy-tale building complex is a strange mixture of emotional pain and anxiety, gratitude and love. It is Valley Children’s Hospital in Madera, California, and it is woven deeply into the story of our lives. Beginning in 2007, Valley Children’s, about one hour from our home, is where I have taken Evren, who has acid sphingomyelinase deficiency, or ASMD, for emergency care and for follow-ups at one of our eleven needed specialty clinics ranging from audiology to pulmonology.

Yet strangely enough, now in 2022, we find ourselves in exile. The time has come that we can no longer set foot inside Valley Children’s doors to find our anxiety dispelled by the dazzling artwork throughout the hospital. It’s a best-kept secret that the hospital doubles as a world-class art gallery whose hallways and exam rooms are adorned with colourful, child-friendly framed pieces, mosaics and murals and three-dimensional works, usually grouped by theme, like fish or farm animals. No longer will we be visiting the hospital’s Starbucks for the welcome distraction of a custom-made cup of coffee, eat chicken strips in the Jellyfish Café after a fasting blood draw or drop notes of thanks to our favourite providers. At twenty, my son has aged out of care at Valley Children’s in both endocrinology and ophthalmology, and it is time to say goodbye to the hospital where we have spent hundreds of hours in a state of heightened emotion.

But how do you say goodbye and find closure when the relationship is not with a person but with dozens of care providers from across a fifteen-year-period? How do we find the courage to let go of this well-known haven we love to avoid and learned to trust, and then reconcile ourselves with our newly exiled status?

In my mind’s eye I can relive Valley Children’s memories from over a decade ago with perfect recall because of “memory-cam”, which switches on during periods of heightened emotion, particularly during emergencies. My head is filled with fifteen years of these recordings. They include the moments I would hear of a newly diagnosed problem, like a severe high-frequency hearing loss or an aortic valve leak, a treatment plan—or lack thereof because nothing could be done—and moments when I watched my child with pinched lips stoically enduring yet another scary medical test, or quietly hearing more bad news—or good news—about his body. My memory recordings feature little brother Erol coming to visit his big brother Evren in the hospital, riding in a little red wagon with IV pole in tow, and playing bingo games at the children’s playroom. I can still feel the relief of respite from an oppressive and depressive state of mind with a joyful visit from the therapy dogs.

During our fifteen years there, most of Evren’s lifetime, we have been served by many physicians and nurses. Most all of them are terrific people, with just a very few exceptions. One such exception was our first geneticist, whom I sought out for help understanding Evren’s symptoms that no one else with ASMD seemed to have, such as joint contractures (loss of range of movement) and vision problems.

Our new geneticist’s alter ego was Magnum P.I. (the 80s’ Tom Selleck version, complete with moustache, clothing style and part-time residence in Hawaii), though I hasten to add his clinical skills were sound, and he has long since moved on from that hospital. Very quickly I could see that we were not going to be a good fit for each other. Although he allowed me to ask a few questions, he was the star of the show, and he really did not want to invest in us beyond our appointment minutes. We ended up severing the relationship with the geneticist right along with the one with our first endocrinologist, new to the specialty, who made two serious mistakes with Evren’s care (she also no longer works there). But most of the physicians were kind professionals who gave us the expert care and time we needed. Our gratitude to them is profound, and they are all written into our book of life.

Then there was the slightly more humorous interaction with a nurse, albeit a little dark, which Evren and I still revisit today. We both vividly recall the time Evren went in for a cortisol and growth hormone test, which requires fasting and having blood drawn every thirty minutes for about four hours while seated in a chair. That certainly was not Evren’s idea of a good time, so he went in rather cranky and developed a classic ASMD headache early on.

When it came time to insert the IV, the young, very gentle and fair-haired male nurse broke out in a terrible sweat when we asked him to insert the needle in Evren’s hand rather than his arm. (Evren’s thickened skin makes the veins in his arms too painful to access). The nurse could sense Evren’s anxiety and tried to begin distraction techniques, but unfortunately the nurse chose the wrong topic. “Do you like to swim?” he asked in a whispery-soft voice. “Do you like the water?” “Do you like to swim in the water?” But the nurse didn’t bother listening to Evren’s best attempts at polite and respectful replies, that no, he did not like the water because he couldn’t swim. 

I just stood back and kept my mouth shut as the nurse was clearly panicked trying to figure out how to find the right vein for the job. He was trying his best. But about the fourth or fifth time he asked if Evren liked to swim in the water, Evren lost it and shouted, “NO! I. CAN’T. SWIM!” The traumatised nurse left at that point and found someone else to take over. And today, when Evren and I realise someone is not listening to us, we roll our eyes at each other, raise our eyebrows with exaggerated height and ask, “Do you like to swim in the water?!”

But on the opposite end of the spectrum, I can still see in my mind’s eye another male nurse. He stepped in with great skill and caring to save an IV that had been inserted days before, so Evren would not have to have another poke with a new IV needle. 

I also hold in my thoughts the genetic counsellor who quietly went the extra mile to see if he couldn’t help us find our way into a compassionate use programme for an enzyme replacement therapy treatment not yet approved in the US.

And then there was Bob, our brilliant physical therapist. Bob was there for Evren year after year, always thinking up new ways to find fun, motivating approaches to combat Evren’s stiff joints and contractures. He treated Evren like a friend, always calm, professional, yet kind and funny. He once made a promise that if Evren could meet his flexibility goals, he would run around the entire Fresno State University campus (which he did—and ended up having to run really fast after a coyote began chasing him at the outer edge of the campus!). We will love Bob forever.

In early 2021, I started seeing signs that the time had come to consider moving on from the children’s hospital. First, it was the way that Evren had to squeeze and fold himself into the tiny paediatric cabinet used while testing lung functioning. In mid-2021, as I was parking the car, the hospital security guard approached Evren to ask him if he had lost track of his child, thinking Evren was a parent, not a patient. 

Finally, at the end of 2021, I observed with great resentment and concern that Evren was being left to languish in the ice-cold emergency room late into the night while suffering from complications of the flu as the children running around the emergency room, laughing and happy, were prioritised due to their young age. After finally being admitted, his body seemed to be too large for the paediatric emergency room bed. I knew in my heart that night that it was time to say goodbye.

I had hoped that the end of our story with Valley Children’s could have ended with enzyme replacement therapy treatment, and Evren and I even made our best attempt to make it happen, but it was not meant to be. It left me feeling empty just to walk away from Valley Children’s without some kind of closure. Evren was just quiet but knew deep down we had to let go. 

In fact, we could have squeezed out another year or two in certain specialties like cardiology or gastroenterology, but for us it is better to avoid keeping one foot in two worlds, the child’s world and the adult’s world, and our HMO insurance plan wouldn’t allow it anyway. Establishing care with new specialty providers, four of which are rather urgent, has only begun and will take dedicated effort and patience as we rebuild trust in a new team of physicians and nurses. It must be done, and so it will be.

But just last week, after three solid days of research and phones calls, we were able to find a promising physician on our plan who agreed to become Evren’s new primary care provider in Fresno. SCORE! As I hung up the phone, I felt the sudden urge to break out some bubbly drink and eat some cake. I suddenly felt as though both my son and I had actually graduated. It was our private graduation day. 

I looked at Evren (seated next to me as he did when we wrote our book) and asked him, “Are you ready to go to a man’s hospital?” 

“Okay,” he agreed with his typical level of mustered optimism and slightly fatalistic tone. My son will persevere. We both know another medical journey awaits, but that is consistent with my definition of life: constant adaptation to constant change. But I am at peace now, having processed my own farewell to our esteemed children’s hospital, no longer welcoming us, but forever a part of our story. Goodbye, Valley Children’s, and thank you. 

Kara and Evren’s book, Extraordinary! A Book for Children with Rare Diseases was published in 2021.

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