My daughter Maureen (Mo) has Loeys-Dietz Syndrome, a connective tissue disease similar to Marfan syndrome. Loeys-Dietz syndrome is complicated. It affects the connective tissue that runs throughout the body—organs, muscles, and blood vessels. You name it. It’s present. When the tissue is weakened and defective, it is bound to wear down, be stretched to its limits and sometimes break. A major issue for Loeys-Dietz patients is they develop aneurysms throughout the body along with having many issues with the joints and other soft tissues.
Mo was born in 1995. Loeys-Dietz syndrome was not discovered until 2005. She has made many trips to Children’s Hospital of Wisconsin since she was born, visiting a number of clinics including orthopedics, ENT and neurology. Her first visit to the Herma Heart Center was in the fall of 2004 when her first aneurysm was diagnosed. It was the final piece to a very large puzzle that we had been working on for nine or so years.
As with many families who have a long-term relationship with Children’s, this has become a second residence for Mo and our family. In one sense, it was home even before Mo was born because I worked in Children’s Emergency Department as a senior nursing student when Children’s was located on 17th and Wisconsin Avenue. After graduating from Marquette University, I took a job on the baby floor, which eventually formed two units in the new hospital – the NICU and the Infant floor. Early on, I learned a critical foundation to excellent nursing care is based in family-centered care – respect, partnership, communication and collaboration with the family. However, returning to my old unit as a mother rather than as a part of the staff bolstered that foundation even more. I never thought I’d become a parent, really a patient, of the very place I worked.
It can be a stressful thing being a nurse and a mother of a chronically, sometimes, critically ill child. My registered nurse license is a great shield to protect my mothering wounds in times of high stress. While I find it a blessing that I received a great nursing education in college and in my hospital employment, there are times I wish I could be the parent that has no understanding of a medical situation, if just for a moment. Over the years the nurses and physicians and entire staff at Children’s have allowed me to be both a nurse and a parent, discussing the diagnosis at hand on one level, while providing a shoulder to lean upon when I’m too tired to carry on. Children’s is our extended family.
Mark your calendar
The Dave & Carole Miracle Marathon is a 36 hour radiothon benefitting the many programs at Children’s Hospital of Wisconsin. Listen to me and Mo tell our story during the radiothon on Thursday, May 24.
Read more of our story
I invite you to read an excerpt from a book I have been working on since Mo was born almost 17 years ago. She’s holding at 26 surgeries and recently passed a two-year anniversary without any surgery, a first ever in her lifetime to go that long between surgeries.
My husband, my daughter, and I fill the small, encased holding area of the surgical department. There has been a modest attempt to transform the institutionalized feel of this space with a small framed photo of babies dressed as cherubs above the head of the gurney. A colorful fabric border depicting children skipping and riding bikes runs along the edge where the walls and ceiling meet. The pale, striped canvas privacy curtain hangs motionless between our space and the bustling nurses’ station outside the OR. We’ve been here many times. Twenty times previously, to be exact. We await the green light for twelve-year old Mo to be moved back to the large operating room. There several specialists will repair the ballooning segments of the main arteries that feed her hardworking heart.
Mo’s eyes roll and her head lolls above her narrow shoulders. The preoperative dose of bitter tasting Versed has made its way through her blood stream and now is transforming her muscles to gelatin. Each movement she makes is overstated and misjudged. Words straggle out of her mouth followed by a sloppy and lusty giggle. Soon these exaggerated antics will cease and her color will pale when the general anesthesia overwhelms her fragile frame, allowing the surgeons to unlock her chest cavity to expose an unwell heart.
The anesthesiologist arrives to examine Mo and once again identify the hazards of this next surgical procedure. His hands palpate her long neck and curved protruding spine. He wants to make sure that my daughter’s ninety-pound, five-foot-six-inch frame will weather the OR table for the next ten hours. Without much natural padding on Mo’s body, he worries about the scarlet sores that may appear from the long periods of pressure. He also voices concern about how far he will flex her head backward before it becomes unsafe. Mo has a congenital defect in her neck that could pose danger to her brain stem and spinal cord if her head is cocked too far back. Putting a breathing tube in requires that the head be positioned like this in order to get correct tube placement.
My daughter has become quieter and less animated. She clutches the hand-stitched edges of her guardian angel quilt that accompanies her to each surgical procedure and hospital stay. When the nurses and doctors announce that the time has arrived to move into the OR, her face blanches. She is instructed to take off her Marquette Steve Novak jersey that she has pulled over her disinfected hospital gown so it will not be lost during the surgery. Mo’s grasp on the blanket swiftly moves to the periphery of her basketball jersey.
“I don’t want to take off my ‘Steve’ jersey. I need to wear it in there,” she insists.
“I’m sorry I am upsetting you, Mo,” the anesthesiologist begins. “I just don’t want you to lose it in there.”
After a bit of back-and-forth bargaining, the anesthesiologist enforces his decision and her tears pour, accompanied by weakened whimpers.
“Mom, we’ll give you a minute to help her take it off.”
Her last piece of control in this whole mess is being stolen from her, and she hasn’t the wits or the strength to combat it any more. Brian and the rest of the patiently waiting team step aside as the pale-striped privacy curtain is pulled.
Mo’s sobbing intensifies as hiccups make their way into the composition of her grief. As I gently tug the nylon fabric over her head, she grabs for my hand. It is cool to the touch, but strong as she tightens her grip on my warm and sweaty palm.
“Mama?” she asks in a much younger and quivering voice. “I don’t want to die. I am so scared.”
My trembling heart lodges in my throat. The jersey falls to the floor in a heap. My arms lock tight around my child’s shuddering frame. My lips press against her damp forehead as my tears sprinkle her soft blond tresses. I am at a loss as to how to comfort my child. My fear mirrors hers, yet I cannot allow myself to reveal it to her.
“W-Would praying help?” I stutter.
She nods in acceptance.
“How about the Hail Mary? It’s one of your favorites. You know Mary is always near us.”
There is a moment of calm before a rush of tears and hushed weeping.
“I can’t remember how to say it,” she confesses.
I say it for the both of us while cradling her against my chest, cupping her head in one trembling hand as my other drapes over her curved and frail shoulder. I have had the honor of holding her this close so many times in the past. I silently implore for this gift to continue in the future. The curtain pulls open and I reluctantly relinquish my embrace.
This is a glimpse into my life for the past 12-plus years and just the latest in a series of gut-wrenching and faith-challenging farewells.
~ Kate H. Jurgens, BSN, RN, prenatal care coordinator, Children’s Community Health Plan